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COST OF TREATMENT AND DIAGNOSTICS OF CANCER IN TURKEY

Lymphoma is a broad term for cancer that begins in the cells of the lymphatic system. The two main types are Hodgkin's lymphoma and non-Hodgkin's lymphoma (NHL). Hodgkin's lymphoma can often be cured. The NHL prognosis depends on the specific type. Explore the links on this page to learn more about lymphoma treatments, research, and clinical trials.

Hodgkin's lymphoma in adults is a disease in which malignant (cancerous) cells form in the lymphatic system.

Hodgkin's lymphoma in adults is a type of cancer that develops in the lymphatic system. The lymphatic system is part of the immune system. This helps protect the body from infections and disease.

The lymphatic system consists of the following:

  • Lymph  : a colorless watery liquid that moves through the lymphatic vessels and carries T- and  B-lymphocytes.  Lymphocytes are white blood cells.

  • Lymphatic Vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.

  • Lymph Nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infections and disease. Lymph nodes are located along a network of lymphatic vessels throughout the body. Groups of lymph nodes are found in the mediastinum (the area between the lungs), neck, armpits, abdomen,  pelvis and  groin. Hodgkin's lymphoma most often forms in the lymph nodes above the diaphragm and often in the lymph nodes of the mediastinum.

  • Spleen  : an organ that produces lymphocytes, stores red blood cells and lymphocytes, filters blood and destroys old blood cells. The spleen is located on the left side of the abdomen next to the belly.

  • Thymus  : the organ in which T-lymphocytes mature and multiply. The thymus is located in the chest behind the sternum.

  • Bone marrow  : soft, spongy tissue in the center of certain bones, such as the femur and sternum. Leukocytes, red blood cells, and platelets are produced in the bone marrow.

  • Tonsils: Two small formations of lymphatic tissue at the back of the pharynx. The tonsils are located on each side of the throat. Hodgkin's lymphoma in adults rarely forms in the tonsils.

1. TREATMENT OF LYMPHOMA IN ADULTS

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Лимфома у взрослых

Anatomy of the lymphatic system showing lymphatic vessels and lymphatic organs including lymph nodes, tonsils, thymus, spleen, and bone marrow. Lymph (clear fluid) and lymphocytes travel through the lymphatic vessels to the lymph nodes, where the lymphocytes destroy harmful substances. Lymph enters the bloodstream through a large vein near the heart.

Lymphatic tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchi, and skin.

There are two main types of lymphoma: Hodgkin's lymphoma and non-Hodgkin's lymphoma. This is a summary of the treatment of Hodgkin's lymphoma in adults, including during pregnancy.

The two main types of Hodgkin's lymphoma are classic lymphoma with a predominance of nodular lymphocytes.

Most Hodgkin's lymphomas are of the classic type. When a sample of lymph node tissue is viewed under a microscope, cancer cells in Hodgkin's lymphoma, called Reed-Sternberg cells, can be seen. The classic type is classified into the following four subtypes:

  • Nodal sclerosing Hodgkin's lymphoma.

  • Mixed cellularity of Hodgkin's lymphoma.

  • Hodgkin's lymphocytic lymphoma.

  • Classic Hodgkin's lymphoma with a high lymphocyte count.

 

Lymphocyte-dominated Hodgkin's lymphoma is rare and tends to grow more slowly than classic Hodgkin's lymphoma. Lymphocyte-dominated Hodgkin's lymphoma often presents with swollen lymph nodes in the neck, chest, armpit, or groin. Most people do not have any other signs or symptoms of cancer at the time of diagnosis. Treatment often differs from classic Hodgkin's lymphoma.

1.1. Risks of developing lymphoma

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean you will get cancer; the absence of risk factors does not mean that you will not get cancer. Talk to your doctor if you think you are at risk. Risk factors for developing Hodgkin's lymphoma in adults include the following:

  • Age . Hodgkin's lymphoma is most common in early adulthood (ages 20–39) and late adulthood (ages 65 and older).

  • Be a man . The risk of adult Hodgkin's lymphoma in men is slightly higher than in women.

  • Past Epstein-Barr virus infection. Infection with the Epstein-Barr virus during adolescence or early childhood increases the risk of Hodgkin's lymphoma.

  • A family history of Hodgkin's lymphoma. Having a parent, sibling, or sister with Hodgkin's lymphoma increases your risk of developing Hodgkin's lymphoma.

1.2. Symptoms of Hodgkin's lymphoma

These and other signs and symptoms can be caused by Hodgkin's lymphoma in adults or other conditions. Talk to your doctor if you have any of the following symptoms that persist:

  • Painless swollen lymph nodes in the neck, armpits, or groin.

  • Fever for no known reason.

  • Wet night sweats.

  • Weight loss for no known reason in the last 6 months.

  • Itching, especially after bathing or drinking alcohol.

  • Feeling very tired.

 

Unknown cause fever, unknown cause weight loss and severe night sweats are called B symptoms. B symptoms are an important part of staging Hodgkin's lymphoma and understanding the patient's chance of recovery.

1.3. Diagnosis and staging of Hodgkin's lymphoma

The results of the tests and procedures below will also help you decide on treatment.

These tests may include:

1. Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness such as tumors or anything else that seems unusual. The patient's health history will also be recorded, including fever, profuse night sweats and weight loss, illness and treatment.

2. Complete blood count (CBC): A procedure in which a blood sample is taken and checked for the following:

  • The number of erythrocytes, leukocytes and platelets.

  • The amount of hemoglobin (oxygen-carrying protein) in red blood cells.

  • The portion of the sample composed of red blood cells.

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Complete blood count (CBC). Blood is collected by inserting a needle into a vein and allowing blood to flow into the tube. A blood sample is sent to a laboratory to count red blood cells, white blood cells, and platelets. A complete blood count is used to test, diagnose, and monitor many different conditions.

3. Studies of the chemical composition of blood  : A procedure in which a blood sample is tested to measure the amount of certain substances that enter the bloodstream by organs and tissues of the body. An unusual (more or less than usual) amount of a substance may be a sign of illness.

4. LDH test: A procedure in which a blood sample is tested to measure the amount of lactate dehydrogenase (LDH). An increased amount of LDH in the blood may be a sign of tissue damage, lymphoma, or other medical conditions.

5. Hepatitis B and Hepatitis C Test: A procedure in which a blood sample is tested to measure the amount of antigens and / or antibodies specific to the hepatitis B virus and the amount of antibodies specific to the hepatitis C virus. These antigens or antibodies are called markers. Various markers or combinations of markers are used to determine if a patient has a hepatitis B or C infection, has had a prior infection or vaccination, or is susceptible to infection. Knowing whether a patient has hepatitis B or C can help plan treatment.

6. HIV test: a test to measure the level of antibodies to the human immunodeficiency virus (HIV) in a blood sample. Antibodies are produced by the body when a foreign substance invades it. A high level of antibodies to HIV can mean that the body is infected with HIV. Knowing if a patient has HIV can help plan treatment.

7. Sedimentation rate: a procedure in which a blood sample is taken and the rate at which red blood cells settle to the bottom of the tube is checked. The settling rate is a measure of how much inflammation is in the body. An excess settling rate may be a sign of lymphoma or other medical conditions. Also called erythrocyte sedimentation rate, sedimentation rate, or ESR.

8. PET / CT scan: A procedure that combines positron emission tomography (PET) and computed tomography (CT) images. PET and CT scans are performed simultaneously on the same machine. Images from both scans are combined to create a more detailed picture than either test could do on its own. PET-CT scans can be used to diagnose a disease such as cancer, determine the stage, plan treatment, or find out if a treatment is effective.

  • Computed tomography (computed tomography): A procedure in which a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, pelvis, and lymph nodes, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed so that organs and tissues can be seen more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography. If a PET-CT scan is not available, only computed tomography can be done.

  • PET scan (Positron Emission Tomography): A PET scan is a procedure to detect malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Cancer cells appear brighter because they are more active and consume more glucose than normal cells.

9. Lymph node biopsy: removal of all or part of the lymph node. A pathologist examines tissue under a microscope for the presence of cancer cells called Reed-Sternberg cells. Reed-Sternberg cells are common in classic Hodgkin's lymphoma.

Диагностика лимфомы

Reed-Sternberg cell. Reed-Sternberg cells are large, abnormal lymphocytes that can contain more than one nucleus. These cells are found in Hodgkin's lymphoma.

One of the following biopsies can be done:

  • Excisional biopsy: Removal of the entire lymph node.

  • Postoperative biopsy: removal of part of the lymph node.

  • Primary biopsy: Removal of tissue from a lymph node using a wide needle.

 

Other areas of the body, such as the liver, lungs, bones, bone marrow, and marrow, may also have a tissue sample taken and checked by a pathologist for signs of cancer.

 

The following test can be performed on the removed tissue:

  • Excisional biopsy: Removal of the entire lymph node.

  • Postoperative biopsy: removal of part of the lymph node.

  • Primary biopsy: Removal of tissue from a lymph node using a wide needle.

10. Immunophenotyping:  a laboratory test that uses antibodies to identify cancer cells based on the types of antigens or markers on the cell surface. This test is used to diagnose certain types of lymphoma.

This test can be performed on the removed tissue.

11. For pregnant women with Hodgkin's lymphoma, imaging techniques are used to protect the unborn baby from the harmful effects of radiation. This includes:

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (MRI). In pregnant women, no contrast dye is used during the procedure.

  • Ultrasound examination: A procedure in which high energy sound waves (ultrasound) are reflected off internal tissues or organs and create an echo. The echo forms a picture of body tissue called a sonogram.

After Hodgkin's lymphoma is diagnosed in adults, tests are done to find out if cancer cells have spread in the lymphatic system or to other parts of the body.

The process used to determine if cancer has spread in the lymphatic system or to other parts of the body is called staging. The information gathered during the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of tests and procedures performed to diagnose and stage Hodgkin's lymphoma are used to make treatment decisions.

Cancer spreads through the body in three ways.

Cancer can spread through tissues, the lymphatic system, and the blood:

  • Textile.  Cancer spreads from where it originated to surrounding areas.

  • The lymphatic system.  Cancer spreads from where it originated to the lymphatic system. Cancer spreads through the lymphatic vessels to other parts of the body.

  • Blood.  Cancer spreads from where it originated into the bloodstream. Cancer spreads through the blood vessels to other parts of the body.

1.4. Factors affecting prognosis and treatment options for Hodgkin's lymphoma

The prognosis and treatment option depends on the following conditions:

  • The patient's signs and symptoms, including B-symptoms (fever for no known cause, weight loss for no known cause, or severe night sweats).

  • The stage of the cancer (the size of the cancerous tumors and the spread of the cancer to the abdomen or more than one group of lymph nodes).

  • A type of Hodgkin's lymphoma.

  • Blood test results.

  • Age, gender and general condition of the patient.

  • Whether the cancer was first diagnosed, continues to grow during treatment, or has returned after treatment.

 

Treatment options for Hodgkin's lymphoma during pregnancy also depend on:

  • Patient wishes.

  • Age of the unborn child.

 

Adult Hodgkin's lymphoma can usually be cured if detected and treated early.

Hodgkin's lymphoma in adults can be grouped for treatment as follows:

 

1. Early favorable

Early favorable Hodgkin's lymphoma in adults is stage I or stage II, with no risk factors that increase the likelihood that the cancer will return after treatment.

2. Early adverse

Early adverse Hodgkin's lymphoma in adults is stage I or stage II with one or more of the following risk factors that increase the likelihood that cancer will return after treatment:

  • The presence of a tumor in the breast that is more than 1/3 of the width of the breast or at least 10 centimeters.

  • Cancer of an organ other than the lymph nodes.

  • Having a high sedimentation rate (in the blood sample, the red blood cells settle to the bottom of the tube faster than usual).

  • Having three or more lymph nodes with cancer.

  • Group B symptoms (fever for unknown cause, weight loss for unknown cause, or severe night sweats).

 

3. Advanced

Advanced Hodgkin's lymphoma is stage III or IV. Advanced favorable Hodgkin's lymphoma means that the patient has 0–3 of the following risk factors. Progressive Adverse Hodgkin's Lymphoma means that the patient has 4 or more of the risk factors listed below. The more risk factors a patient has, the more likely the cancer will return after treatment:

  • Low level of albumin (protein) in the blood (below 4).

  • Low hemoglobin levels (below 10.5).

  • Be a man.

  • Age 45 and older.

  • Stage IV disease.

  • Having a high white blood cell count (15,000 or higher).

  • Low lymphocyte count (less than 600 or less than 8% of the white blood cell count).

1.5. Stages of Hodgkin's lymphoma in adults

Stage I

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Adult stage I lymphomas. Cancer is found in one or more lymph nodes in a group of lymph nodes or, in rare cases, cancer is found in Waldeyer's ring, thymus, or spleen. In stage IE (not shown), the cancer has spread to one area outside the lymphatic system.

Stage I adult Hodgkin's lymphoma is divided into stages I and IE.

  • In stage I, cancer is found in one of the following areas of the lymphatic system:

    • One or more lymph nodes in a group of lymph nodes.

    • Waldeyer's ring.

    • Thymus.

    • Spleen.

  • In stage IE, cancer is found in one area outside the lymphatic system.

 

Stage II

Stage II adult Hodgkin's lymphoma is subdivided into stage II and IIE.

  • In stage II, cancer is found in two or more groups of lymph nodes located either above the diaphragm or below the diaphragm.

2 стадия лимфомы Ходжкина

Stage II adult lymphomas. Cancer is found in two or more groups of lymph nodes located either above the diaphragm or below the diaphragm.

In stage IIE, the cancer has spread from a group of lymph nodes to a nearby area that is outside the lymphatic system.  The cancer may have spread to other groups of lymph nodes on the same side of the diaphragm.

2 Е стадия лимфомы Ходжкина

Adult stage IIE lymphomas. The cancer has spread from a group of lymph nodes to a nearby area that is outside the lymphatic system. The cancer may have spread to other groups of lymph nodes on the same side of the diaphragm.

In stage II, the term "bulky disease" refers to a larger tumor.  The size of the tumor mass, called bulk disease, depends on the type of lymphoma.

 

Stage III

3 стадия лимфомы Ходжкина

Stage III adult lymphomas. Cancer is found in groups of lymph nodes both above and below the diaphragm; or in a group of lymph nodes above the diaphragm and in the spleen.

Stage III adult Hodgkin's lymphoma,  cancer occurs:

  • in groups of lymph nodes both above and below the diaphragm; or

  • in the lymph nodes above the diaphragm and in the spleen.

 

Stage IV

4 стадия лимфомы Ходжкина

Stage IV adult lymphomas. Cancer (a) has spread to one or more organs outside the lymphatic system; or (b) is found in two or more groups of lymph nodes that are either above the diaphragm or below the diaphragm, and in one organ that is outside the lymphatic system and not near the affected lymph nodes; or (c) is found in groups of lymph nodes above the diaphragm and below the diaphragm and in any organ outside the lymphatic system; or (d) is found in the liver, bone marrow, more than one location in the lungs, or cerebrospinal fluid (CSF). The cancer has not spread directly to the liver, bone marrow, lungs, or cerebrospinal fluid from nearby lymph nodes.

Stage IV adult Hodgkin's lymphoma, cancer:

  • Has spread to one or more organs outside the lymphatic system or

  • is found in two or more groups of lymph nodes that are either above the diaphragm or below the diaphragm, and in one organ that is outside the lymphatic system, and not next to the affected lymph nodes; or

  • found in groups of lymph nodes both above and below the diaphragm, as well as in any organ outside the lymphatic system; or

  • is found in the liver, bone marrow, more than one place in the lungs, or cerebrospinal fluid (CSF). The cancer has not spread directly to the liver, bone marrow, lungs, or cerebrospinal fluid from nearby lymph nodes.

1.6. Overview of treatment options

 

KEY POINTS

  • There are various treatments for patients with Hodgkin's lymphoma in adults.

  • For patients with Hodgkin's lymphoma, treatment should be planned by a team of healthcare professionals experienced in treating lymphomas.

  • Treatment for Hodgkin's lymphoma in adults can cause side effects.

  • Four types of standard treatments are used:

    • Chemotherapy

    • Radiation therapy

    • Targeted therapy

    • Immunotherapy

  • For pregnant women with Hodgkin's lymphoma, treatment options also include:

    • Watchful waiting

    • Steroid therapy

  • New treatments are undergoing clinical trials.

    • Stem Cell Transplant Chemotherapy

  • Patients may want to take part in a clinical trial.

  • Patients can participate in clinical trials before, during, or after starting cancer treatment.

  • Additional tests may be required.

 

There are various treatments for patients with Hodgkin's lymphoma in adults.

 

Various treatments are available for patients with adult Hodgkin's lymphoma. Some treatments are standard (currently used) and some are in clinical trials. A treatment clinical trial is a scientific study designed to help improve existing treatments or provide information about new treatments for cancer patients. When clinical trials show that a new treatment is better than the standard treatment, the new treatment can become the standard treatment. Patients may want to take part in a clinical trial. Some clinical trials are only open to patients who have not yet started treatment.

For pregnant women with Hodgkin's lymphoma, treatment is carefully tailored to protect the unborn baby. Treatment decisions depend on the mother's wishes, the stage of Hodgkin's lymphoma, and the age of the unborn child. Your treatment plan may change as the signs and symptoms of cancer and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family and healthcare staff.

For patients with Hodgkin's lymphoma, treatment should be planned by a team of healthcare professionals experienced in treating lymphomas.

Treatment will be overseen by an oncologist, a doctor who specializes in cancer treatment. Your medical oncologist may refer you to other health care providers who have experience and expertise in treating Hodgkin's lymphoma in adults and specialize in specific areas of medicine. This may include the following specialists:

  • Radiologist oncologist.

  • Rehabilitation specialist.

  • Hematologist.

  • Other specialists are oncologists.

1.7. Side effects after treatment

 

Treatment for Hodgkin's lymphoma in adults can cause side effects.

 

Side effects from cancer treatments that start after treatment and last for months or years are called late effects. Treating Hodgkin's lymphoma with chemotherapy and / or radiation therapy may increase the risk of recurring cancer and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the age of the patient at the time of treatment and may include the following:

  • Second cancer.

    • Acute myeloid leukemia and non-Hodgkin's lymphoma.

    • Solid tumors such as mesothelioma and cancer of the lungs, breast, thyroid, bones, soft tissues, stomach, esophagus, colon, rectum, cervix, and head and neck.

  • Infertility.

  • Hypothyroidism (too little thyroid hormone in the blood).

  • Heart disease, such as a heart attack.

  • Lung problems such as shortness of breath.

  • Avascular bone necrosis (death of bone cells due to lack of blood flow).

  • Severe infection.

  • Chronic fatigue .

 

Regular follow-up by physicians who are experts in detecting and treating late effects is important for the long-term health of patients being treated for Hodgkin's lymphoma.

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2. TREATMENT OF NONHODGKIN'S LYMPHOMA IN ADULTS

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Non-Hodgkin's lymphoma  is a type of cancer that forms in the lymphatic system. The lymphatic system is part of the immune system. This helps protect the body from infections and disease.

The lymphatic system consists of the following:

  • Lymph: A colorless, watery fluid that travels through the lymphatic vessels and carries lymphocytes (white blood cells). There are three types of lymphocytes:

    • B lymphocytes, which produce antibodies that help fight infection. Also called B cells. Most types of non-Hodgkin's lymphoma begin with B cells.

    • T lymphocytes, which help B lymphocytes make antibodies that help fight infection. Also called T-lymphocytes.

    • Natural killer cells attacking cancer cells and  viruses. Also called NK cells.

  • Lymphatic Vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.

  • Lymph Nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infections and disease. Lymph nodes are located along a network of lymphatic vessels throughout the body. Lymph node groups are found in the neck, armpits, mediastinum, abdomen, pelvis, and groin.

  • Spleen:  an organ that produces lymphocytes, stores red blood cells and lymphocytes, filters blood and destroys old blood cells. The spleen is located on the left side of the abdomen next to the belly.

  • Thymus: The organ in which T-lymphocytes mature and multiply. The thymus is located in the chest behind the sternum.

  • Tonsils: Two small formations of lymphatic tissue at the back of the pharynx. The tonsils are located on each side of the throat.

  • Bone marrow: The soft, spongy tissue in the center of certain bones, such as the femur and sternum. Leukocytes, red blood cells, and platelets are produced in the bone marrow.

лимфатическая ситстема из
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Anatomy of the lymphatic system showing lymphatic vessels and lymphatic organs including lymph nodes, tonsils, thymus, spleen, and bone marrow. Lymph (clear fluid) and lymphocytes travel through the lymphatic vessels to the lymph nodes, where the lymphocytes destroy harmful substances. Lymph enters the bloodstream through a large vein near the heart.

Lymphatic tissue is also found in other parts of the body, such as the lining of the digestive tract, bronchi, and skin. Cancer can spread to the liver and lungs.

There are two main types of lymphomas:  Hodgkin's lymphoma  and non-Hodgkin's lymphoma. This is a summary of the treatment for non-Hodgkin's lymphoma in adults, including during pregnancy.

2.1. NHL types

Non-Hodgkin's lymphoma grows and spreads at different rates and can be sluggish or aggressive. Painless lymphoma tends to grow and spread slowly and has few signs and symptoms. Aggressive lymphoma grows and spreads rapidly, and its signs and symptoms can be very serious. Treatments for indolent and aggressive lymphoma are different.

 

This is a brief description of the following types of non-Hodgkin's lymphoma:

Indolent non-Hodgkin lymphomas

  • Follicular lymphoma. Follicular lymphoma is the most common type of indolent non-Hodgkin's lymphoma. It is a very slowly growing type of non-Hodgkin's lymphoma that begins with B cells. It affects the lymph nodes and can spread to the bone marrow or spleen. Most patients with follicular lymphoma are 50 years of age or older at the time of diagnosis. Follicular lymphoma can go away without treatment. The patient is closely monitored for signs or symptoms of disease recurrence. Treatment is necessary if signs or symptoms appear after the cancer has gone away or after initial cancer treatment. Sometimes follicular lymphoma can become a more aggressive type of lymphoma, such as diffuse large B-cell lymphoma.

  • Lymphoplasmacytic lymphoma. In most cases of lymphoplasmacytic lymphoma, B lymphocytes, which convert to plasma cells, produce large amounts of a protein called monoclonal immunoglobulin M (IgM) antibody. A high level of IgM antibodies in the blood leads to a thickening of the blood plasma. It can cause signs or symptoms such as vision or hearing problems, heart problems, shortness of breath, headache, dizziness, and numbness or tingling in the hands and feet. Sometimes there are no signs or symptoms of lymphoplasmacytic lymphoma. This can be found in the blood test for another reason. Lymphoplasmacytic lymphoma often spreads to the bone marrow, lymph nodes, and spleen. Patients with lymphoplasmacytic lymphoma should be tested for hepatitis C virus infection. This is also called Waldenstrom's macroglobulinemia.

  • Lymphoma of the marginal zone. This type of non-Hodgkin's lymphoma begins with B cells in a part of the lymphatic tissue called the marginal zone. The prognosis may be worse for patients age 70 or older, stage III or stage IV disease, and high lactate dehydrogenase (LDH) levels. There are five different types of marginal zone lymphoma. They are grouped by the type of tissue in which the lymphoma has formed:

    • Marginal zone nodular lymphoma. In the lymph nodes, nodular lymphoma of the marginal zone is formed. This type of non-Hodgkin's lymphoma is rare. It is also called monocytoid B-cell lymphoma.

    • Lymphoma associated with the gastric mucosa, lymphoid tissue (MALT). MALT gastric lymphoma usually begins in the stomach. This type of marginal lymphoma forms in the cells of the mucous membrane that help make antibodies. Patients with gastric MALT lymphoma may also have Helicobacter gastritis or an autoimmune disease such as Hashimoto's thyroiditis or Sjogren's syndrome.

    • Extra-gastric lymphoma MALT. Extra-gastric MALT lymphoma begins outside the stomach in almost all parts of the body, including other parts of the gastrointestinal tract, salivary glands, thyroid gland, lungs, skin, and around the eyes. This type of marginal lymphoma forms in the cells of the mucous membrane that help make antibodies. Extra-gastric MALT lymphoma may return many years after treatment.

    • Mediterranean lymphoma of the abdominal cavity. It is a type of MALT lymphoma that occurs in young people in the Eastern Mediterranean countries. It often forms in the abdominal area and patients can also become infected with a bacteria called Campylobacter jejuni. This type of lymphoma is also called immunoproliferative disease of the small intestine.

    • Lymphoma of the marginal zone of the spleen. This type of marginal lymphoma begins in the spleen and can spread to peripheral blood and bone marrow. The most common sign of this type of marginal splenic lymphoma is the spleen, which is larger than usual.

  • Primary cutaneous anaplastic large cell lymphoma. This type of non-Hodgkin's lymphoma affects only the skin. It may be a benign (non-cancer) nodule that may go away on its own, or it may have spread to many areas of the skin and require treatment.

 

Aggressive non-Hodgkin lymphomas

  • Large diffuse B-cell lymphoma. Diffuse large B cell lymphoma is the most common type of non-Hodgkin's lymphoma. It grows rapidly in the lymph nodes, and often the spleen, liver, bone marrow, or other organs are also affected. Signs and symptoms of diffuse large B cell lymphoma can include fever, severe night sweats, and weight loss. They are also called B symptoms.

    • Primary large B-cell lymphoma of the mediastinum. This type of non-Hodgkin's lymphoma is a type of diffuse large B-cell lymphoma. The proliferation of fibrous (cicatricial) lymphatic tissue is noted. Tumors most often form behind the sternum. It can press on the airways and cause coughing and shortness of breath. Most patients with primary large B-cell lymphoma of the mediastinum are women between the ages of 30 and 40.

  • Follicular large cell lymphoma stage III. Stage III follicular large cell lymphoma is a very rare type of non-Hodgkin's lymphoma. Treatment for this type of follicular lymphoma is more like treating aggressive NHL than indolent NHL.

  • Anaplastic large cell lymphoma. Anaplastic large cell lymphoma is a type of non-Hodgkin's lymphoma that usually begins in T lymphocytes. Cancer cells also have the CD30 marker on the cell surface.

     

There are two types of anaplastic large cell lymphoma:

1 . Cutaneous anaplastic large cell lymphoma. This type of anaplastic large cell lymphoma primarily affects the skin, but other parts of the body can also be affected. Signs of cutaneous anaplastic large cell lymphoma include one or more bumps or sores on the skin. This type of lymphoma is rare and sluggish.

2. Systemic anaplastic large cell lymphoma. This type of anaplastic large cell lymphoma begins in the lymph nodes and can affect other parts of the body. This type of lymphoma is more aggressive. Patients may have a lot of anaplastic lymphoma kinase (ALK) protein inside lymphoma cells. These patients have a better prognosis than those who do not have the supplemental ALK protein. Systemic anaplastic large cell lymphoma is more common in children than in adults. (For more information, see the PDQ Treatment Summary for Pediatric Non-Hodgkin's Lymphoma.)

  • Extranodal NK- / T-cell lymphoma. Extranodal NK / T cell lymphoma usually begins in the area around the nose. It can also affect the paranasal sinuses (cavities in the bones around the nose), palate, trachea, skin, stomach, and intestines. In most cases of extranodal NK- / T-cell lymphoma, the Epstein-Barr virus is present in the tumor cells. Sometimes hemophagocytic syndrome (a serious condition in which there are too many active histiocytes and T cells causing severe inflammation in the body) occurs. Treatment is needed to suppress the immune system. This type of non-Hodgkin's lymphoma is not common in the United States.

  • Lymphatic granulomatosis. Lymphomatoid granulomatosis most commonly affects the lungs. It can also affect the sinuses (cavities in the bones around the nose), skin, kidneys, and the central nervous system. In lymphomatoid granulomatosis, cancer attacks the blood vessels and kills tissue. Because cancer can spread to the brain, intrathecal chemotherapy or radiation therapy to the brain is given.

  • Angioimmunoblastic T-cell lymphoma. This type of non-Hodgkin's lymphoma starts with T cells. Swollen lymph nodes are a common symptom. Other signs may include a skin rash, fever, weight loss, or severe night sweats. There may also be high levels of gamma globulin (antibodies) in the blood. Patients can also have opportunistic infections because their immune systems are weakened.

  • Peripheral T-cell lymphoma. Peripheral T-cell lymphoma begins with mature T-lymphocytes. This type of T lymphocyte matures in the thymus gland and travels to other lymphatic regions of the body, such as the lymph nodes, bone marrow, and spleen. There are three subtypes of peripheral T-cell lymphoma:

    • Hepatosplenic T-cell lymphoma. This is an uncommon type of peripheral T-cell lymphoma that occurs mainly in young men. It starts in the liver and spleen, and cancer cells also have a T cell receptor called gamma / delta on the cell's surface.

    • Subcutaneous panniculitis-like T-cell lymphoma. Subcutaneous panniculitis-like T-cell lymphoma begins on the skin or mucous membrane. This can happen with hemophagocytic syndrome (a serious condition in which there are too many active histiocytes and T cells that cause severe inflammation in the body). Treatment is needed to suppress the immune system.

    • Intestinal T-cell lymphoma of the enteropathic type. This type of peripheral T-cell lymphoma occurs in the small intestine of patients with untreated celiac disease (an immune response to gluten that causes malnutrition). Patients diagnosed with celiac disease in childhood and who follow a gluten-free diet rarely develop enteropathic-type intestinal T-cell lymphoma.

  • Intravascular large B-cell lymphoma. This type of non-Hodgkin's lymphoma affects the blood vessels, especially the small blood vessels in the brain, kidneys, lungs, and skin. The signs and symptoms of intravascular large B-cell lymphoma are caused by blockage of blood vessels. It is also called intravascular lymphomatosis.

  • Burkitt's lymphoma  ... Burkitt's lymphoma is a type of B-cell non-Hodgkin's lymphoma that grows and spreads very quickly. It can affect the jaw, facial bones, intestines, kidneys, ovaries, or other organs. There are three main types of Burkitt's lymphoma (endemic, sporadic, and immunodeficient). Endemic Burkitt's lymphoma is commonly found in Africa and is associated with the Epstein-Barr virus, while sporadic Burkitt's lymphoma occurs throughout the world. Burkitt's lymphoma, associated with immunodeficiency, is most common in people with AIDS. Burkitt's lymphoma can spread to the brain and spinal cord and requires treatment to prevent it from spreading. Burkitt's lymphoma is most common in children and young adults. Burkitt's lymphoma is also called diffuse small cell lymphoma.

  • Lymphoblastic lymphoma. Lymphoblastic lymphoma can start in T cells or B cells, but it usually starts in T cells. In this type of non-Hodgkin's lymphoma, there are too many lymphoblasts (immature white blood cells) in the lymph nodes and thymus gland. These lymphoblasts can spread to other parts of the body, such as the bone marrow, brain, and spinal cord. Lymphoblastic lymphoma is most common in adolescents and young adults. This is very similar to acute lymphoblastic leukemia (lymphoblasts are mainly found in the bone marrow and blood). (For more information, see the PDQ Treatment Summary for Acute Lymphoblastic Leukemia in Adults.)

  • Adult T cell leukemia / lymphoma. Adult T cell leukemia / lymphoma is caused by human T cell leukemia virus type 1 (HTLV-1). Signs include bone and skin lesions, high blood calcium levels, and larger than normal lymph nodes, spleen, and liver.

  • Mantle cell lymphoma. Mantle cell lymphoma is a type of B-cell non-Hodgkin's lymphoma that commonly occurs in middle-aged and older people. It begins in the lymph nodes and spreads to the spleen, bone marrow, blood, and sometimes to the esophagus, stomach, and intestines. Patients with mantle cell lymphoma have too much of a protein called cyclin-D1, or certain gene changes in lymphoma cells. In some patients who do not have signs or symptoms of lymphoma, delaying treatment initiation does not affect prognosis.

  • Post-transplant lymphoproliferative disease. This disease occurs in patients who have undergone heart, lung, liver, kidney, or pancreas transplants and require lifelong immunosuppressive therapy. Most post-transplant lymphoproliferative diseases involve B cells and contain the Epstein-Barr virus in the cells. Lymphoproliferative disorders are often treated like cancer.

  • True histiocytic lymphoma. This is a rare, very aggressive type of lymphoma. It is not known whether it starts in B cells or T cells. He is difficult to treat with standard chemotherapy.

  • Primary effusion lymphoma. Primary effusion lymphoma begins with B cells, which are found in areas of large accumulation of fluid, such as the areas between the lining of the lung and the chest wall (pleural effusion), the sac around the heart, and the heart. (pericardial effusion) or abdominal cavity. Usually, the tumor is not visible. This type of lymphoma is common in HIV-infected patients.

  • Plasmablastic lymphoma  ... Plasmablastic lymphoma is a very aggressive type of large cell non-Hodgkin's lymphoma. Most often seen in patients with HIV infection.

2.2. NHL Symptoms

Signs and symptoms of non-Hodgkin's lymphoma in adults include swollen lymph nodes, fever, profuse night sweats, weight loss, and fatigue.

 

These signs and symptoms can be caused by adult non-Hodgkin's lymphoma or other medical conditions. Check with your doctor if you have any of the following:

  • Swelling of the lymph nodes in the neck, armpits, groin, or abdomen.

  • Fever for no known reason.

  • Wet night sweats.

  • Feeling very tired.

  • Losing weight for an unknown reason.

  • Skin rash or itching.

  • Pain in the chest, abdomen, or bones for no known cause.

 

When fever, wet night sweats, and weight loss occur together, this group of symptoms is called symptom B.

Other signs and symptoms of non-Hodgkin's lymphoma in adults may occur, depending on the following:

  • Where cancer forms in the body.

  • The size of the tumor.

  • How quickly the tumor grows.

2.3. Tests for  diagnosis and staging of non-Hodgkin's lymphoma in adults

The following tests and procedures can be used:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness such as bumps or anything else that seems out of the ordinary. The patient's health history will also be recorded, including fever, profuse night sweats and weight loss, his health habits, and any illnesses and treatments he has suffered.

  • Complete blood count (CBC): A procedure in which a blood sample is taken and checked for the following:

    • The number of erythrocytes, leukocytes and platelets.

    • The amount of hemoglobin (oxygen-carrying protein) in red blood cells.

    • The portion of the sample composed of red blood cells.

  • Blood chemistry tests: A procedure in which a blood sample is tested to measure the amount of certain substances that enter the bloodstream by organs and tissues of the body. An unusual (more or less than usual) amount of a substance may be a sign of illness.

  • LDH test: A procedure in which a blood sample is tested to measure the amount of lactodehydrogenase. An increased amount of LDH in the blood may be a sign of tissue damage, lymphoma, or other medical conditions.

  • Hepatitis B and Hepatitis C Test: A procedure in which a blood sample is tested to measure the amount of antigens and / or antibodies specific to the hepatitis B virus and the amount of antibodies specific to the hepatitis C virus. These antigens or antibodies are called markers. Various markers or combinations of markers are used to determine if a patient has a hepatitis B or C infection, has had a prior infection or vaccination, or is susceptible to infection. Patients who have been treated for the hepatitis B virus in the past need constant monitoring to see if it has reactivated. Knowing whether a person has hepatitis B or C can help plan treatment.

  • HIV test: A test to measure the level of HIV antibodies in a blood sample. Antibodies are produced by the body when a foreign substance invades it. A high level of antibodies to HIV can mean that the body is infected with HIV.

  • CT (Computed Tomography): A procedure in which a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, pelvis, and lymph nodes, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs and tissues more visible. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • PET scan (positron emission tomography) : a procedure to search for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Cancer cells appear brighter because they are more active and consume more glucose than normal cells.

  • Bone marrow aspiration and biopsy: Removal of bone marrow and a small piece of bone by inserting a needle into the hip or sternum. A pathologist examines the bone marrow and bones under a microscope to look for signs of cancer.

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Bone marrow aspiration and biopsy. After a small area of skin is numb, a bone marrow needle is inserted into the patient's hip bone. Samples of blood, bone, and bone marrow are taken for examination under a microscope.

  • Lymph node biopsy: Removal of all or part of a lymph node. A pathologist examines the tissue under a microscope to check for cancer cells. One of the following biopsies can be done:

    • Excisional biopsy: Removal of the entire lymph node.

    • Postoperative biopsy: removal of part of the lymph node.

    • Primary biopsy: Removal of part of the lymph node with a wide needle.

 

When cancer is found, the following tests can be performed to look at cancer cells :

  • Immunohistochemistry:  a laboratory test that uses antibodies to check for specific antigens (markers) in a tissue sample from a patient. Antibodies are usually associated with an enzyme or fluorescent dye. After antibodies bind to a specific antigen in a tissue sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test is used to diagnose cancer and help distinguish one type of cancer from another.

  • Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a blood or bone marrow sample are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in some chromosomes can be a sign of cancer. Cytogenetic analysis is used to diagnose cancer, plan treatment, or determine the effectiveness of a treatment.

  • Immunophenotyping: A laboratory test that uses antibodies to identify cancer cells based on the types of antigens or markers on the cell surface. This test is used to diagnose certain types of lymphoma.

  • FISH (Fluorescence In Situ Hybridization): A laboratory test used to study and count genes or chromosomes in cells and tissues. DNA fragments containing fluorescent dyes are made in the laboratory and added to a sample of the patient's cells or tissues. When these stained DNA fragments attach to specific genes or regions of chromosomes in a sample, they light up when viewed under a fluorescent microscope. The FISH test is used to diagnose cancer and plan treatment.

 

Other tests and procedures may be performed depending on the signs and symptoms observed and where the cancer is forming in the body.

2.4. Factors affecting prognosis (chance of recovery) and treatment options

Prognosis and treatment options depend on the following:

  • The patient's signs and symptoms, including B-symptoms (fever for no known cause, weight loss for no known cause, or severe night sweats).

  • The stage of the cancer (the size of the cancerous tumors and whether the cancer has spread to other parts of the body or lymph nodes).

  • A type of non-Hodgkin's lymphoma.

  • The amount of lactate dehydrogenase (LDH) in the blood.

  • Are there any changes in the genes.

  • Age, gender and general condition of the patient.

  • Whether lymphoma is a new diagnosis, continues to grow during treatment, or recurs (comes back).

 

Treatment options for non-Hodgkin's lymphoma during pregnancy also depend on:

  • Patient wishes.

  • What trimester of pregnancy is the patient in?

  • Is it possible to give birth to a child ahead of time.

 

Some types of non-Hodgkin's lymphoma spread faster than others. Most non-Hodgkin lymphomas that occur during pregnancy are aggressive. Delaying treatment for aggressive lymphoma until the baby is born can lower the mother's chances of survival. Immediate treatment is often recommended, even during pregnancy.

2.5. NHL stages in adults

KEY POINTS

  • After adults are diagnosed with non-Hodgkin's lymphoma, tests are done to see if cancer cells have spread in the lymphatic system or to other parts of the body.

  • Cancer spreads through the body in three ways.

  • For adult non-Hodgkin lymphoma, the following stages are used:

    • Stage I

    • Stage II

    • Stage III

    • Stage IV

  • Adult non-Hodgkin's lymphomas can be grouped for treatment depending on whether the cancer is indolent or aggressive, whether the affected lymph nodes are close to each other in the body, and whether the cancer is newly diagnosed or recurrent.

 

After adults are diagnosed with non-Hodgkin's lymphoma, tests are done to see if cancer cells have spread in the lymphatic system or to other parts of the body.

The process used to identify the type of cancer and the spread of cancer cells in the lymphatic system or to other parts of the body is called staging. The information gathered during the staging process determines the stage of the disease. It is important to know the stage of the disease in order to plan treatment. The results of tests and procedures performed to diagnose non-Hodgkin's lymphoma are used to make treatment decisions.

The following tests and procedures can also be used in the preparation process:

  • Gadolinium MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body, such as the brain and spinal cord. A substance called gadolinium is given to the patient through a vein. Gadolinium gathers around cancer cells, so they appear brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spine. This is done by inserting a needle between two bones in the spine and into the cerebrospinal fluid and taking a sample of the fluid. A sample of spinal fluid is checked under a microscope for signs that cancer has spread to the brain and spinal cord. This procedure is also called LP or lumbar puncture.

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спинномозговая пункция

Lumbar puncture. The patient lies on the table, curled up in a ball. After a small area in the lower back is numb, a spinal needle (long, thin needle) is inserted into the lower spinal column to remove cerebrospinal fluid (cerebrospinal fluid, shown in blue). Liquid can be sent​​ to the laboratory for testing.

For pregnant women with non-Hodgkin's lymphoma, interim tests and procedures are used to protect the unborn baby from the harmful effects of radiation. These tests and procedures include MRI (no contrast), lumbar puncture, and ultrasound.

Cancer spreads through the body in three ways.

Cancer can spread through tissues, the lymphatic system, and the blood:

  • Textile. Cancer spreads from where it originated to surrounding areas.

  • The lymphatic system. Cancer spreads from where it originated to the lymphatic system. Cancer spreads through the lymphatic vessels to other parts of the body.

  • Blood. Cancer spreads from where it originated into the bloodstream. Cancer spreads through the blood vessels to other parts of the body.

For adult non-Hodgkin lymphoma, the following stages are used:

Stage I

1 стадия Неходжкинской лимфомы

Adult stage I lymphomas. Cancer is found in one or more lymph nodes in a group of lymph nodes or, in rare cases, cancer is found in Waldeyer's ring, thymus, or spleen. In stage IE (not shown), the cancer has spread to one area outside the lymphatic system.

Stage I adult non-Hodgkin's lymphoma is divided into stage I and IE.

In stage I, cancer is found in one of the following areas of the lymphatic system:

  • One or more lymph nodes in a group of lymph nodes.

  • Waldeyer's ring.

  • Thymus.

  • Spleen.

 

In stage IE, cancer is found in one area outside the lymphatic system.

 

Stage II

Stage II adult non-Hodgkin's lymphoma is divided into stage II and IIE.

  • In stage II, cancer is found in two or more groups of lymph nodes located either above the diaphragm or below the diaphragm.

2 стадия Неходжкинской лимфомы

In stage IIE, the cancer has spread from a group of lymph nodes to a nearby area that is outside the lymphatic system.  The cancer may have spread to other groups of lymph nodes on the same side of the diaphragm.

In stage II, the term "volumetric disease" refers to a greater  tumors  ... The size of the tumor mass, called volumetric disease, depends on the type  lymphomas  ...

 

Stage III

3 стадия лимфоны Неходжкина

Stage III adult lymphomas. Cancer is found in groups of lymph nodes both above and below the diaphragm; or in a group of lymph nodes above the diaphragm and in the spleen.

Stage III adult non-Hodgkin lymphoma,  cancer occurs:

  • in groups of lymph nodes both above and below the diaphragm; or

  • in the lymph nodes above the diaphragm and in the spleen.

 

Stage IV

4 стадия лимфомы Неходжкина

Stage IV adult lymphomas. Cancer (a) has spread to one or more organs outside the lymphatic system; or (b) is found in two or more groups of lymph nodes that are either above the diaphragm or below the diaphragm, and in one organ that is outside the lymphatic system and not near the affected lymph nodes; or (c) is found in groups of lymph nodes above the diaphragm and below the diaphragm and in any organ outside the lymphatic system; or (d) is found in the liver, bone marrow, more than one location in the lungs, or cerebrospinal fluid (CSF). The cancer has not spread directly to the liver, bone marrow, lungs, or cerebrospinal fluid from nearby lymph nodes.

Stage IV adult non-Hodgkin lymphoma, cancer:

  • Has spread to one or more organs outside the lymphatic system or

  • is found in two or more groups of lymph nodes that are either above the diaphragm or below the diaphragm, and in one organ that is outside the lymphatic system, and not next to the affected lymph nodes; or

  • found in groups of lymph nodes both above and below the diaphragm, as well as in any organ outside the lymphatic system; or

  • is found in the liver, bone marrow, more than one place in the lungs, or cerebrospinal fluid (CSF). The cancer has not spread directly to the liver, bone marrow, lungs, or cerebrospinal fluid from nearby lymph nodes.

 

Adult non-Hodgkin's lymphomas can be grouped for treatment depending on whether the cancer is indolent or aggressive, whether the affected lymph nodes are close to each other in the body, and whether the cancer is newly diagnosed or recurrent.

Non-Hodgkin's lymphoma can also be described as contiguous or non-contiguous:

  • Adjacent lymphomas: Lymphomas in which lymph nodes with cancer are located next to each other.

  • Non-contiguous lymphomas: Lymphomas in which lymph nodes with cancer are not located next to each other, but on one side of the diaphragm.

 

Recurrent non-Hodgkin's lymphoma in adults is cancer that has recurred (returned) after treatment. Lymphoma can return to the lymphatic system or to other parts of the body. Painless lymphoma can return to aggressive lymphoma. Aggressive lymphoma can return to lazy lymphoma.

2.6. Overview of NHL Treatment Options

KEY POINTS

  • There are various treatments for patients with non-Hodgkin's lymphoma.

  • For patients with non-Hodgkin's lymphoma, treatment should be planned by a team of healthcare professionals who are experts in the treatment of lymphomas.

  • Treatment for non-Hodgkin's lymphoma in adults can cause side effects.

  • Nine types of standard treatments are used:

    • Radiation therapy

    • Chemotherapy

    • Immunotherapy

    • Targeted therapy

    • Plasmapheresis

    • Watchful waiting

    • Antibacterial therapy

    • Operation

    • Stem cell transplant

  • New treatments are undergoing clinical trials.

    • Vaccine therapy

  • Patients may want to take part in a clinical trial.

  • Patients can participate in clinical trials before, during, or after starting cancer treatment.

  • Additional tests may be required.

 

2.7. Treatments for patients with non-Hodgkin's lymphoma

Various treatments are available for patients with non-Hodgkin lymphoma. Some treatments are standard (currently used) and some are in clinical trials. A treatment clinical trial is a scientific study designed to help improve existing treatments or provide information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment can become the standard treatment. Patients may want to take part in a clinical trial. Some clinical trials are only open to patients who have not yet started treatment.

For pregnant women with non-Hodgkin's lymphoma, treatment is carefully chosen to protect the unborn baby. Treatment decisions depend on the mother's wishes, the stage of non-Hodgkin's lymphoma, and the age of the unborn child. Your treatment plan may change as the signs and symptoms of cancer and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family and healthcare staff.

For patients with non-Hodgkin's lymphoma, treatment should be planned by a team of healthcare professionals who are experts in the treatment of lymphomas.

Your treatment will be overseen by an oncologist, a doctor who specializes in cancer treatment, or a hematologist, a doctor who specializes in treating blood cancer. Your medical oncologist may refer you to other health care providers who are experienced and expert in treating non-Hodgkin's lymphoma in adults and specialize in specific areas of medicine. This may include the following specialists:

  • Neurosurgeon.

  • Neurologist.

  • Radiologist oncologist.

  • Endocrinologist.

  • Rehabilitation specialist.

  • Other specialists are oncologists.

 

2.8. Side effects of NHL treatment

 

Treatment for non-Hodgkin's lymphoma in adults can cause side effects.

 

Side effects from cancer treatments that start after treatment and last for months or years are called late effects. Treating non-Hodgkin's lymphoma with chemotherapy, radiation therapy, or stem cell transplantation may increase the risk of late effects.

Late effects of cancer treatments may include the following:

 

Some late effects can be treated or controlled. It is important to talk to your doctor about the effects of cancer treatments on you. Regular follow-up is important to detect late effects.

2.9. Standard treatments

Nine types of standard treatments are used:

1. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy X-rays or other types of radiation to kill cancer cells or prevent them from growing.

External beam radiation therapy uses a device outside the body to direct radiation to an area of the body affected by cancer. Sometimes the whole body is irradiated before stem cell transplantation.

Proton beam radiation therapy is a type of high-energy external beam therapy that uses beams of protons (tiny particles with a positive charge) to destroy tumor cells. This type of treatment can reduce the level of radiation damage to healthy tissues near the tumor, such as the heart or chest.

External beam radiation therapy is used to treat non-Hodgkin's lymphoma in adults and can also be used as palliative therapy to relieve symptoms and improve quality of life.

Pregnant women with non-Hodgkin's lymphoma should receive radiation therapy after delivery, if possible, to avoid risk to the unborn baby. If treatment is needed immediately, the woman may decide to continue the pregnancy and receive radiation therapy. A lead shield is used to cover a pregnant woman's belly to help protect the unborn baby from radiation as much as possible.

 

2. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is injected directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ or body cavity such as the abdomen, the drugs mainly target cancer cells in those areas (regional chemotherapy). Combined chemotherapy treatment using two or more anticancer drugs. Steroid medications may be added to reduce inflammation and lower the body's immune response.

Systemic combination chemotherapy is used to treat non-Hodgkin's lymphoma in adults.

Intrathecal chemotherapy can also be used to treat lymphoma that first forms in the testes or sinuses (cavities) around the nose, diffuse large B-cell lymphoma, Burkitt's lymphoma, lymphoblastic lymphoma, and some aggressive T-cell lymphomas. It is prescribed to reduce the likelihood of lymphoma cells spreading to the brain and spinal cord. This is called CNS prophylaxis.

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Интратектальная химиотерапия

Intrathecal chemotherapy. Anti-cancer drugs are injected into the intrathecal space, which is the space that contains the cerebrospinal fluid (cerebrospinal fluid, shown in blue). This can be done in two different ways. One way, shown at the top of the picture, is to inject drugs into the Ommaya reservoir (a domed container that is placed under the scalp during surgery; it holds the drugs as they pass through a small tube to the brain.). Another method, shown at the bottom of the figure, is to inject the drugs directly into the cerebrospinal fluid in the lower spine after a small area in the lower back is numb.

When a pregnant woman is undergoing chemotherapy for non-Hodgkin's lymphoma, the unborn baby cannot be protected from the effects of the chemotherapy. Some chemotherapy regimens can cause birth defects if given in the first trimester.

 

3. Immunotherapy

Immunotherapy  is a treatment that uses the patient's immune system to fight cancer. Substances produced by the body or produced in the laboratory are used to enhance, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biological therapy.

  • Immunomodulators:  lenalidomide is an immunomodulator used to treat non-Hodgkin's lymphoma in adults.

  • CAR T Cell Therapy: The patient's T cells (a type of cells in the immune system) change so they attack certain proteins on the surface of the cancer cells. T cells are taken from the patient, and special receptors are added to their surface in the laboratory. The altered cells are called chimeric antigen receptor (CAR) T cells. CAR T cells are grown in the laboratory and infused into the patient. CAR T cells multiply in the patient's blood and attack cancer cells. CAR T cell therapy (eg axicabtagen ciloleucel or tisagenlekleucel) is used to treat large B cell lymphomas that do not respond to treatment. CAR T-cell therapy is being studied to treat mantle cell lymphoma that has recurred or has not responded to treatment.

 

4. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and target certain cancer cells. Targeted therapy usually does less damage to normal cells than chemotherapy or radiation therapy. Monoclonal antibody therapy, proteasome inhibitor therapy, and kinase inhibitor therapy are the types of targeted therapies used to treat adult non-Hodgkin's lymphoma.

  • Monoclonal antibody therapy: Monoclonal antibodies are immune system proteins created in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that can promote cancer cell growth. The antibodies are then able to kill cancer cells, block their growth, or prevent them from spreading. Monoclonal antibodies are given by infusion. They can be used alone or to carry drugs, toxins, or radioactive materials directly to cancer cells.

    Types of monoclonal antibodies include:

    • Obinutuzumab, used to treat follicular lymphoma.

    • Mogamulizumab, used to treat certain types of recurrent or refractory T-cell lymphoma.

    • Tafasitamab in combination with lenalidomide for the treatment of recurrent or refractory diffuse large B-cell lymphoma.

    • Pembrolizumab for the treatment of primary large B-cell lymphoma of the mediastinum.

    • Polatuzumab vedotin in combination with bendamustine and rituximab for the treatment of recurrent or refractory diffuse large B-cell lymphoma.

    • Brentuximab vedotin, a monoclonal antibody that binds to the CD30 protein found on some lymphoma cells. It also contains an anticancer drug that can help kill cancer cells.

    • Yttrium Y 90-ibritumomab tuxetan, an example of a radiolabeled monoclonal antibody.

    • Rituximab  used to treat many types of non-Hodgkin's lymphoma.

 

  • Proteasome inhibitor therapy: This treatment blocks the action of proteasomes in cancer cells. Proteasomes remove proteins that the cell no longer needs. When proteasomes are blocked, proteins build up in the cell and can cause cancer cell death. Bortezomib is used to reduce the amount of immunoglobulin M in the blood after treatment for cancer of lymphoplasmacytic lymphoma (Waldenstrom's macroglobulinemia). It is also being studied for the treatment of recurrent mantle cell lymphoma.

  • Kinase inhibitor therapy: This treatment blocks certain proteins that can help prevent the growth of lymphoma cells and kill them. Kinase inhibitor therapy includes:

 

  1. Copanlisib, idealisib, dubelisib, and umbralisib, which block P13K proteins and may help prevent lymphoma cell growth. They are used to treat follicular non-Hodgkin's lymphomas that have recurred (returned) or have not improved after treatment with at least two other treatments. Umbralisib is also used to treat marginal zone lymphoma that has recurred or has not improved after treatment.

  2. Ibrutinib, acalabrutinib, and zanubrutinib, which are types of Bruton tyrosine kinase inhibitor therapy. They are used to treat mantle cell lymphoma. Ibrutinib and acalabrutinib are also used to treat lymphoplasmacytic lymphoma, and zanubrutinib is being studied for its treatment.

 

  • Histone methyltransferase inhibitor therapy: Tasemetostat is used to treat follicular lymphoma that has returned or has not improved with other treatments. It is used in adults in whom cancer has a specific mutation (change) in the EZH2 gene that has already been treated with at least two other anticancer drugs.

  • B cell lymphoma-2 (BCL-2) inhibitor therapy: Venetoclax can be used to treat mantle cell lymphoma. It blocks the action of the BCL-2 protein and may help kill cancer cells.

 

5. Plasmapheresis

If the blood becomes thick due to additional antibody proteins and affects circulation, plasmapheresis is done to remove excess plasma and antibody proteins from the blood. In this procedure, blood is drawn from a patient and passed through a machine that separates the plasma (the liquid portion of the blood) from the blood cells. The patient's plasma contains unnecessary antibodies and is not returned to the patient. Normal blood cells are returned to the bloodstream along with donated plasma or plasma substitutes. Plasmapheresis does not prevent the formation of new antibodies.

6. Watchful waiting

Expectant close monitoring of the patient's condition without giving any treatment until signs or symptoms or changes appear.

 

7. Antibiotic therapy

Antibiotic therapy is a treatment that uses medications to treat infections and cancers caused by bacteria and other microorganisms.

 

8. Operation

Surgery may be used to remove lymphoma in some patients with indolent or aggressive non-Hodgkin's lymphoma.

The type of surgery used depends on where the lymphoma originated in the body:

  • Local excision in some patients with mucosal lymphoma, lymphoid (MALT) lymphoma, PTLD, and small bowel T-cell lymphoma.

  • Splenectomy for patients with marginal zone lymphoma from the spleen.

 

Patients who have had heart, lung, liver, kidney, or pancreas transplants usually need to take medications that suppress their immune system for the rest of their lives. Long-term immunosuppression after organ transplantation can cause a specific type of non-Hodgkin's lymphoma called post-transplant lymphoproliferative disorder (PLTD).

 

Small bowel surgery is often needed to diagnose celiac disease in adults who develop a type of T-cell lymphoma.

 

9. Stem cell transplant

Stem cell transplantation is a method of administering high doses of chemotherapy and / or whole-body radiation, followed by replacement of hematopoietic cells destroyed by cancer treatment. Stem cells (immature blood cells) are removed from the patient's blood or bone marrow (autologous transplant) or donor (allogeneic transplant), frozen and stored. After completing chemotherapy and / or radiation therapy, retained stem cells

defrost and return to the patient as an infusion. These re-injected stem cells germinate (and regenerate) the body's blood cells.

пересадка стволовых клеток

Stem cell transplant. (Step 1): Blood is drawn from a vein in the donor's arm. The donor can be a patient or other person. The blood goes through a machine that removes stem cells. The blood is then returned to the donor through a vein in the other arm. (Step 2): The patient receives chemotherapy to destroy the blood-forming cells. The patient may receive radiation therapy (not shown). (Step 3): The patient receives stem cells through a catheter placed in a blood vessel in the chest.

New treatments are undergoing clinical trials.

Vaccine therapy  is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find a tumor and kill it.

COST OF TREATMENT AND DIAGNOSTICS OF LYMPHOMA IN TURKEY

 

  • Immunotherapy with Keytruda (Pembrolizumab) $ 3300 - $ 4700

  • Ultrasound $ 100 - $ 300

  • Blood test for tumor markers $ 150 - $ 500

  • Biopsy $ 1000 - $ 4900

  • MRI of one area $ 100 - $ 1000

  • Immunohistochemistry $ 600 - $ 1000

  • Diagnostics + Chemotherapy from $ 2800

  • Diagnosis for lymphomas + BMT from $ 40,050

  • Comprehensive diagnostics for suspected lymphoma from $ 3800

  • Allogeneic bone marrow transplant from a related donor for lymphoma from $ 60,000

  • Allogeneic bone marrow transplant from an unrelated donor for lymphoma from $ 75,000

  • Autologous bone marrow transplant for lymphoma from $ 37,000

  • Bone marrow transplant for lymphoma from $ 19,900

  • CAR T-cell therapy for lymphoma on request

 

with HODGKIN'S lymphoma:

  • Immunotherapy with Keytruda (Pembrolizumab) for Hodgkin's disease from $ 3300

  • Chemotherapy for breast cancer from $ 1200

  • Radiation therapy for rectal cancer  on request

 

with FOCULAR lymphoma:

  • Complex diagnostics of lymphomas in follicular lymphoma on request

  • Allogeneic bone marrow transplant from a related donor for follicular lymphoma from $ 60,000

  • Allogeneic bone marrow transplant from an unrelated donor for follicular lymphoma from $ 75,000

  • Autologous bone marrow transplant for follicular lymphoma from $ 37,000from 

  • Bone Marrow Transplant for Follicular Lymphoma from $ 19,900

  • Chemotherapy for breast cancer from $ 1200

  • Radiation therapy for rectal cancer on request

 

with NECHODGKIN'S lymphoma:

  • Comprehensive diagnosis of lymphomas in non-Hodgkin's lymphoma on request

  • Allogeneic bone marrow transplant from a related donor for non-Hodgkin's lymphoma from $ 60,000

  • Allogeneic bone marrow transplant from an unrelated donor for non-Hodgkin's lymphoma from $ 75,000

  • Autologous bone marrow transplant for non-Hodgkin's lymphoma from $ 37,000

  • Bone Marrow Transplant for Non-Hodgkin's Lymphoma from $ 19,900

  • Chemotherapy for breast cancer from $ 1200

  • Radiation therapy for rectal cancer on request

with BERKITT's lymphoma:

  • Comprehensive diagnosis of lymphomas in Burkitt's lymphoma on request

  • Allogeneic bone marrow transplant from a related donor for Burkitt's lymphoma from $ 60,000

  • Allogeneic bone marrow transplant from an unrelated donor for Burkitt's lymphoma from $ 75,000

  • Autologous Bone Marrow Transplant for Burkitt's Lymphoma from $ 37,000

  • Bone marrow transplant for Burkitt's lymphoma from $ 19900 Chemotherapy for breast cancer from $ 1200

  • Radiation therapy for rectal cancer on request

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3. TREATMENT OF LYMPHOMA ASSOCIATED WITH  AIDS

Якорь 3

3.1. General information about AIDS-related lymphoma

AIDS-related lymphoma is a disease in which malignant (cancerous) cells form in the lymphatic system of patients with acquired immunodeficiency syndrome (AIDS).

AIDS is caused by the Human Immunodeficiency Virus (HIV), which attacks and weakens the body's immune system. A weakened immune system is unable to fight off infections and diseases. People with HIV infection have an increased risk of infection and lymphoma or other types of cancer. A person with HIV and certain types of infection or cancer, such as lymphoma, is diagnosed with AIDS. Sometimes people are diagnosed with AIDS and AIDS-related lymphoma at the same time.

The lymphatic system consists of the following: (click here )

Lymphomas are divided into two main types : (click here )

3.2. Signs of AIDS-related lymphoma

These and other signs and symptoms can be caused by AIDS-related lymphoma or other conditions. Check with your doctor if you have any of the following:

  • Weight loss or increased body temperature for an unknown reason.

  • Wet night sweats.

  • Painless swollen lymph nodes in the neck, chest, armpits, or groin.

  • Feeling of fullness under the ribs.

3.3. Tests for the diagnosis of AIDS-related lymphoma.

Tests that examine the lymphatic system and other parts of the body are used to diagnose AIDS-related lymphoma.

The following tests and procedures can be used:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness such as bumps or anything else that seems out of the ordinary. The patient's health history will also be recorded, including fever, profuse night sweats and weight loss, health habits, and medical and medical history.

  • Complete blood count (CBC): A procedure in which a blood sample is taken and checked for the following:

    • The number of erythrocytes, leukocytes and platelets.

    • The amount of hemoglobin (oxygen-carrying protein) in red blood cells.

    • The portion of the sample composed of red blood cells.

  • Blood chemistry tests: A procedure in which a blood sample is tested to measure the amount of certain substances that enter the bloodstream by organs and tissues of the body. An unusual (more or less than usual) amount of a substance may be a sign of illness.

  • LDH test: A procedure in which a blood sample is tested to measure the amount of lactodehydrogenase. An increased amount of LDH in the blood may be a sign of tissue damage, lymphoma, or other medical conditions.

  • Hepatitis B and Hepatitis C Test: A procedure in which a blood sample is tested to measure the amount of antigens and / or antibodies specific to the hepatitis B virus and the amount of antibodies specific to the hepatitis C virus. These antigens or antibodies are called markers. Various markers or combinations of markers are used to determine if a patient has a hepatitis B or C infection, has had a prior infection or vaccination, or is susceptible to infection.

  • HIV test: A test to measure the level of HIV antibodies in a blood sample. Antibodies are produced by the body when a foreign substance invades it. A high level of antibodies to HIV can mean that the body is infected with HIV.

  • CT (Computed Tomography): A procedure in which a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, pelvis, and lymph nodes, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs and tissues more visible. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • PET scan (positron emission tomography): a procedure to look for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Cancer cells appear brighter because they are more active and consume more glucose than normal cells.

  • Bone marrow aspiration and biopsy: Removal of bone marrow and a small piece of bone by inserting a cannula into the hip or sternum. A pathologist examines the bone marrow and bones under a microscope to look for signs of cancer.

  • Lymph node biopsy: Removal of all or part of a lymph node. A pathologist examines tissue under a microscope for cancer cells. One of the following biopsies can be done:

  1. Excisional biopsy: Removal of the entire lymph node.

  2. Postoperative biopsy: removal of part of the lymph node.

  3. Primary biopsy: Removal of tissue from a lymph node using a wide needle.

 

Other areas of the body, such as the liver, lungs, bones, bone marrow, and marrow, may also have a tissue sample taken and checked by a pathologist for signs of cancer.

When cancer is found, the following tests can be performed to look at cancer cells:

  • Immunohistochemistry:  a laboratory test that uses antibodies to check for specific antigens (markers) in a tissue sample from a patient. Antibodies are usually associated with an enzyme or fluorescent dye. After antibodies bind to a specific antigen in a tissue sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test is used to help diagnose cancer and to help distinguish one type of cancer from another.

  • Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a blood or bone marrow sample are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in some chromosomes can be a sign of cancer. Cytogenetic analysis is used to diagnose cancer, plan treatment, or determine the effectiveness of a treatment.

  • FISH (Fluorescence In Situ Hybridization): A laboratory test used to study and count genes or chromosomes in cells and tissues. Pieces of DNA containing fluorescent dyes are made in the laboratory and added to a sample of the patient's cells or tissues. When these stained DNA fragments attach to specific genes or regions of chromosomes in a sample, they light up when viewed under a fluorescent microscope. The FISH test is used to diagnose cancer and plan treatment.

  • Immunophenotyping: A laboratory test that uses antibodies to identify cancer cells based on the types of antigens or markers on the cell surface. This test is used to diagnose certain types of lymphoma.

After AIDS-related lymphoma is diagnosed, tests are done to see if cancer cells have spread in the lymphatic system or to other parts of the body.

The process used to determine if cancer cells have spread in the lymphatic system or to other parts of the body is called staging. The information gathered during the staging process determines the stage of the disease. It is important to know the stage to plan treatment, but AIDS-related lymphoma is usually in an advanced stage when diagnosed.

The following tests and procedures can be used to find out if the cancer has spread:

  • Gadolinium MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of the interior of the body, such as the brain and spinal cord. A substance called gadolinium is given to the patient through a vein. Gadolinium gathers around cancer cells, so they appear brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spine. This is done by placing a needle between two bones in the spine and into the cerebrospinal fluid and taking a sample of the fluid. A sample of spinal fluid is checked under a microscope for signs that cancer has spread to the brain and spinal cord. The sample can also be tested for the Epstein-Barr virus. This procedure is also called LP or lumbar puncture.

3.4. Factors affecting prognosis (chance of recovery) and treatment options

The prognosis and treatment option depends on the following conditions:

  • Stage of cancer.

  • The age of the patient.

  • The number of CD4 lymphocytes (a type of white blood cell) in the blood.

  • A number of places in the body of lymphoma are outside the lymphatic system.

  • Whether the patient has a history of intravenous (IV) drug use.

  • The patient's ability to carry out normal daily activities.

3.5. Overview of treatment options

KEY POINTS

  • There are various treatments for patients with AIDS-related lymphoma.

  • Treatment for AIDS-related lymphoma combines treatment for lymphoma with treatment for AIDS.

  • Four types of standard treatments are used:

    • Chemotherapy

    • Radiation therapy

    • High-dose chemotherapy with stem cell transplant

    • Targeted therapy

  • New treatments are undergoing clinical trials.

  • Treatment for AIDS-related lymphoma can cause side effects.

  • Patients may want to take part in a clinical trial.

  • Patients can participate in clinical trials before, during, or after starting cancer treatment.

  • Additional tests may be required.

For treatment, AIDS-related lymphomas are grouped according to where they originated in the body, as follows:

  • Peripheral / systemic lymphoma

Lymphoma that begins in the lymphatic system or elsewhere in the body besides the brain is called peripheral / systemic lymphoma. It can spread throughout the body, including the brain or bone marrow. It is often diagnosed at an advanced stage.

Treatment for AIDS-related peripheral / systemic lymphoma may include the following:

  • Combined chemotherapy with or without targeted therapy.

  • High-dose chemotherapy and stem cell transplant for lymphoma that has not responded to treatment or has returned.

  • Intrathecal chemotherapy for lymphoma, which can spread to the central nervous system (CNS).

  • Primary CNS lymphoma

Primary CNS lymphoma begins in the central nervous system (brain and spinal cord). It is associated with the Epstein-Barr virus. Lymphoma that starts elsewhere in the body and spreads to the central nervous system is not primary CNS lymphoma.

Treatment for AIDS-related primary central nervous system lymphoma may include the following:

  • External beam therapy.

Treatment for AIDS-related lymphoma combines treatment for lymphoma with treatment for AIDS.

AIDS patients have a weakened immune system, and treatment may cause the immune system to become weaker. For this reason, treatment of patients who have AIDS-related lymphoma is difficult and some patients may be treated with lower doses of medication than patients with lymphomas who do not have AIDS.

Highly active antiretroviral therapy (HAART) is used to reduce the damage to the immune system caused by HIV. HAART treatment may allow some patients with AIDS-related lymphoma to safely receive anti-cancer drugs at standard or higher doses. In these patients, treatment may work just as well as in patients with lymphoma who do not have AIDS. Medicines are also used to prevent and treat infections that can be serious.

Four types of standard treatments are used:

 

1. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is injected directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ or body cavity such as the abdomen, the drugs mainly target cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment that uses more than one anticancer drug.

Intrathecal chemotherapy can be used in patients who are more likely to have central nervous system (CNS) lymphoma.

Chemotherapy is used in the treatment of AIDS-related peripheral / systemic lymphoma. It is not yet known whether it is better to administer HAART at the same time as chemotherapy or after chemotherapy ends.

Colony-stimulating factors are sometimes given in conjunction with chemotherapy. This helps reduce the side effects that chemotherapy can have on the bone marrow.

2. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy X-rays or other types of radiation to kill cancer cells or prevent them from growing. External beam radiation therapy uses a device outside the body to direct radiation to an area of the body affected by cancer.

3. High-dose chemotherapy with stem cell transplantation

High doses of chemotherapy are prescribed to kill cancer cells. Healthy cells, including hematopoietic cells, are also destroyed in cancer treatments. Stem cell transplantation is a treatment to replace hematopoietic cells. Stem cells (immature blood cells) are removed from the patient's blood or bone marrow, frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and returned to the patient as an infusion. These re-injected stem cells germinate (and regenerate) the body's blood cells.

4. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to target and attack certain cancer cells without harming normal cells.

  • Monoclonal antibody therapy: This treatment uses antibodies obtained in the laboratory from one type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that can promote the growth of cancer cells. Antibodies attach to substances and kill cancer cells, block their growth, or prevent them from spreading. Monoclonal antibodies are given by infusion. They can be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Rituximab is used to treat AIDS-related peripheral / systemic lymphoma.

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3.2.
3.3.
3.4.
3.5.
якорь 4

4. TREATMENT OF FUNGAL MYCOSIS (including Sesari's syndrome)

Fungal mycosis and Sesary's syndrome are diseases in which lymphocytes (a type of white blood cell) become malignant (cancerous) and affect the skin.

Usually, the bone marrow produces blood stem cells (immature cells), which over time become mature blood stem cells. A blood stem cell can become a myeloid stem cell or a lymphoid stem cell. The myeloid stem cell becomes an erythrocyte,  leukocyte or platelet.  The lymphoid stem cell becomes a lymphoblast and then one of three types of lymphocytes (leukocytes):

  • B-cell lymphocytes, which make antibodies to help fight infection.

  • T lymphocytes, which help B lymphocytes produce antibodies that help fight infection.

  • Natural killer cells that attack cancer cells and viruses.

526538-750.jpg

Development of blood cells. A blood stem cell goes through several stages to become an erythrocyte, platelet, or white blood cell.

Fungal mycosis and Sesary's syndrome are types of cutaneous T-cell lymphoma.

Fungal mycosis and Sesary's syndrome are the two most common types of cutaneous T-cell lymphoma (a type of non-Hodgkin's lymphoma).  

see NHL treatment (click here )

4.1. Symptoms  

1. A sign of fungal mycosis is a red rash on the skin.

Fungal mycosis can go through the following phases:

  • Premycotic phase: a scaly red rash on areas of the body that are not normally exposed to the sun. This rash does not cause symptoms and can last for months or years. At this stage, it is difficult to diagnose the rash as fungal mycosis.

  • Patch phase: Thin, flushed, eczema-like rash.

  • Plaque phase: Small bumps (papules) or hard rashes on the skin that may turn red.

  • Tumor phase: Tumors form on the skin. Ulcers can form in these tumors and the skin can become infected.

 

Check with your doctor if you have any of these signs.

2. With Sesari's syndrome, cancer T cells are found in the blood.

In addition, the skin all over the body was red, itchy, flaky, and painful. There may also be spots, plaques, or swellings on the skin. It is not known whether Sesari's syndrome is an advanced form of fungal mycosis or a separate disease.

4.2. Diagnostics  fungal mycosis and Cesari's syndrome

To diagnose fungal mycosis and Cesari's syndrome, tests are used that examine the skin and blood.

The following tests and procedures can be used:

  • Physical examination and health history: A body exam to check for general signs of health, including checking for signs of illness such as bumps, the number and type of skin lesions, or anything else that seems unusual. Skin scans and a history of the patient's habits as well as past illnesses and treatments will also be taken.

  • Complete blood count with differential: A procedure in which a blood sample is taken and tested for the following:

    • The number of red blood cells and platelets.

    • The number and type of leukocytes.

    • The amount of hemoglobin (oxygen-carrying protein) in red blood cells.

    • The portion of a blood sample made up of red blood cells.

  • Cesari Blood Cell Counting: A procedure in which a blood sample is viewed under a microscope to count the number of Cesari cells.

  • HIV test: A test to measure the level of HIV antibodies in a blood sample. Antibodies are produced by the body when a foreign substance invades it. A high level of antibodies to HIV can mean that the body is infected with HIV.

  • Skin biopsy: Removing cells or tissues so they can be viewed under a microscope for signs of cancer. The doctor may remove the growth from the skin, which will be examined by a pathologist. More than one skin biopsy may be required to diagnose fungal mycosis. Other tests that can be performed on cells or a tissue sample include the following:

  1. Immunophenotyping:  a laboratory test that uses antibodies to identify cancer cells based on the types of antigens or markers on the cell surface. This test is used to diagnose certain types of lymphoma.

  2. Flow cytometry: A laboratory test that measures the number of cells in a sample, the percentage of living cells in the sample, and certain characteristics of cells such as size, shape, and the presence of tumor (or other) markers on the cell surface. Cells from a sample of blood, bone marrow, or other tissue from a patient are stained with a fluorescent dye, placed in a liquid, and then passed one at a time through a beam of light. The test results are based on how cells stained with a fluorescent dye respond to a beam of light. This test is used to diagnose and treat certain types of cancer, such as leukemia and lymphoma.

  • T cell receptor gene rearrangement (TCR) test: A laboratory test that tests cells in a sample of blood or bone marrow to see if there are certain changes in genes that create receptors on T cells (white blood cells). Testing for these gene changes can determine if a large number of T cells are being produced with a specific T cell receptor.

After fungal mycosis and Sesari's syndrome are diagnosed, tests are done to find out if the cancer cells have spread from the skin to other parts of the body.

The process used to determine if cancer has spread from the skin to other parts of the body is called staging. The information gathered during the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.

During the preparation process, the following procedures can be used:

  • Chest X-ray:  X-ray of organs and bones inside the chest. X-rays are a type of energy beam that can pass through the body onto film, creating an image of areas within the body.

  • CT (computed tomography): A procedure in which a series of detailed pictures of areas inside the body, such as the lymph nodes, chest, abdomen, and pelvis, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • PET scan (positron emission tomography): a procedure to look for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Cancer cells appear brighter because they are more active and consume more glucose than normal cells.

  • Lymph node biopsy: Removal of all or part of a lymph node. A pathologist examines the lymph node tissue under a microscope to check for cancerous cells.

  • Bone marrow aspiration and biopsy: Removal of bone marrow and a small piece of bone by inserting a cannula into the hip or sternum. A pathologist examines the bone marrow and bone under a microscope to look for signs of cancer.

4.3 Factors influencing  on prognosis (chance of recovery) and treatment options.

The prognosis and treatment option depends on the following conditions:

  • Stage of cancer.

  • The type of lesion (spots, plaques, or tumors).

  • Age and gender of the patient.

 

Fungal mycosis and Sesari's syndrome are difficult to cure. Treatment is usually palliative to relieve symptoms and improve quality of life. Patients with an early stage of the disease can live for many years.

4.4. Stages at  fungal mycosis and Sesari's syndrome

With fungal mycosis and Sesari's syndrome, the following stages are used:

Fungal mycosis stage I

 

Stage I is divided into stages IA and IB as follows:

  • Stage IA: spots, papules and / or plaques cover less than 10% of the skin surface.

  • Stage IB: spots, papules and / or plaques cover 10% or more of the skin surface.

 

There may be a small number of Cesars  cells  v  blood  ...

 

Fungoid mycosis stage II

Phase II is divided into Phases IIA and IIB as follows:

  • Stage IIA: spots, papules and / or plaques cover any surface of the skin. Lymph nodes are abnormal, but they are not cancerous.

  • Stage IIB: One or more tumors of 1 cm or more are found on the skin. Lymph nodes may be abnormal, but they are not cancerous.

There may be a small number of Cesari cells in the blood.

 

Fungoid mycosis stage III

In stage III, 80% or more of the skin's surface turns red and may have spots, papules, plaques, or tumors. Lymph nodes may be abnormal, but they are not cancerous.

There may be a small number of Cesari cells in the blood.

Fungal mycosis stage IV / Sesari's syndrome

When a large number of Sesari cells are present in the blood, the disease is called Sesari's syndrome.

Stage IV is divided into stages IVA1, IVA2 and IVB as follows:

  • Stage IVA1: spots, papules, plaques, or tumors may cover any part of the skin surface, and 80% or more of the skin surface may be reddened. The lymph nodes may be abnormal, but they are not cancerous. There are a large number of Cesars of cells in the blood.

  • Stage IVA2: spots, papules, plaques, or tumors may cover any part of the skin surface, and 80% or more of the skin surface may be reddened. The lymph nodes are very abnormal, or cancer has formed in the lymph nodes. There may be a large number of Sesari cells in the blood.

  • Stage IVB: Cancer has spread to other organs in the body, such as the spleen or liver. Spots, papules, plaques, or tumors can cover any part of the skin's surface, and 80% or more of the skin's surface may be reddened. Lymph nodes can be abnormal or cancerous. There may be a large number of Sesari cells in the blood.

 

Fungal mycosis and Sesari's syndrome may recur (come back) after treatment.

Fungal mycosis and Sesari's syndrome can recur on the skin or in other parts of the body, such as the spleen or liver.

4.5. Overview of treatment options  

There are various treatments for patients with fungal mycosis and cancer with Sesari's syndrome.

Various treatments are available for patients with fungal mycosis and Sesari's syndrome. Some treatments are standard (currently used) and some are in clinical trials. A clinical trial of treatment is a scientific study designed to help improve current treatments or provide information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than standard treatment, the new treatment may become the standard treatment. Patients may want to take part in a clinical trial. Some clinical trials are only open to patients who have not yet started treatment.

Seven types of standard treatments are used:

 

1. Photodynamic therapy

Photodynamic therapy is a cancer treatment that uses drugs and a certain type of laser beam to kill cancer cells. A drug that is inactive before exposure to light is injected into a vein. The drug accumulates in cancer cells more than in normal cells. In skin cancer, a laser beam hits the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy practically does not damage healthy tissues. Patients undergoing photodynamic therapy should limit their exposure to the sun. There are different types of photodynamic therapy:

  • With psoralen and ultraviolet A (PUVA) therapy, the patient is given a drug called psoralen and then the ultraviolet A light is directed to the skin.

  • In extracorporeal photochemotherapy, drugs are administered to the patient, then some blood cells are taken from the body, placed in a special ultraviolet light A and returned back to the body. Extracorporeal photochemotherapy can be used alone or in combination with total skin electron beam (TSEB) radiation therapy.

 

2. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy X-rays or other types of radiation to kill cancer cells or prevent them from growing. External beam radiation therapy uses a device outside the body to direct radiation to an area of the body affected by cancer. Sometimes, total skin electron beam (TSEB) radiation therapy is used to treat fungal mycosis and Sesari's syndrome. This is a type of external beam treatment in which a radiation therapy machine directs electrons (tiny invisible particles) to the skin that covers the entire body. External beam radiation therapy can also be used as palliative therapy to relieve symptoms and improve quality of life.

Ultraviolet A (UVA) radiation therapy or ultraviolet B (UVB) radiation therapy can be performed using a special lamp or laser that directs radiation to the skin.

3. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). Sometimes chemotherapy is topical (applied to the skin with a cream, lotion, or ointment).

 

4. Other drug therapy

Topical corticosteroids are used to reduce redness, swelling, and inflammation of the skin. This is a type of steroid. Topical corticosteroids can be in the form of a cream, lotion, or ointment.

Retinoids such as bexarotene are vitamin A-related drugs that can slow the growth of certain types of cancer cells. Retinoids can be taken orally or applied to the skin.

Lenalidomide is a medicine that helps the immune system kill abnormal blood cells or cancer cells and can prevent the growth of new blood vessels needed for tumors to grow.

Vorinostat and romidepsin are two histone deacetylase (HDAC) inhibitors used to treat fungal mycosis and Sesari's syndrome. HDAC inhibitors cause a chemical change that stops tumor cells from dividing.

5. Immunotherapy

Immunotherapy is a treatment that uses a patient's immune system to fight cancer. Substances produced by the body or produced in the laboratory are used to enhance, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or biological therapy.

  • Interferon: This treatment prevents fungal mycosis and Sesari cells from dividing and may slow tumor growth.

6. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to target cancer cells. Targeted therapy usually does less damage to normal cells than chemotherapy or radiation therapy.

  • Monoclonal antibody therapy: This treatment uses antibodies obtained in the laboratory from one type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that can promote the growth of cancer cells. Antibodies attach to substances and kill cancer cells, block their growth, or prevent them from spreading. They can be used alone or to carry drugs, toxins, or radioactive materials directly to cancer cells. Monoclonal antibodies are given by infusion.

    Types of monoclonal antibodies include:

    • Brentuximab vedotin, which contains monoclonal antibodies that bind to the CD30 protein found on some types of lymphoma cells. It also contains an anti-cancer drug that can help kill cancer cells.

    • Mogamulizumab, which contains monoclonal antibodies that bind to the CCR4 protein found on certain types of lymphoma cells. It can block this protein and help the immune system kill cancer cells. It is used to treat fungal mycosis and Sesari's syndrome that have returned or failed to improve after treatment with at least one systemic therapy.

 

7. High-dose chemotherapy and radiation therapy with stem cell transplantation

High doses of chemotherapy and sometimes radiation therapy are given to kill cancer cells. Healthy cells, including hematopoietic cells, are also destroyed in cancer treatments. Stem cell transplantation is a treatment to replace hematopoietic cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of a patient or donor, frozen and stored. After the patient completes chemotherapy and radiation therapy, the stored stem cells are thawed and returned to the patient as an infusion. These re-injected stem cells germinate (and regenerate) the body's blood cells.

4.6. Treatment options by stage

Treatment of fungal mycosis stage I and II

 

Treatment for newly diagnosed stage I and II fungal mycoses may include the following:

  • Psoralen and ultraviolet A radiation therapy (PUVA).

  • Ultraviolet radiation In therapy.

  • Radiation therapy with total electron beam therapy of the skin. In some cases, radiation therapy is applied to skin lesions as palliative therapy to reduce tumor size, relieve symptoms, and improve quality of life.

  • Immunotherapy given alone or in combination with skin-targeted therapy.

  • Local chemotherapy.

  • Systemic chemotherapy with one or more drugs that can be combined with skin-directed therapy.

  • Other drug therapy (topical corticosteroids, retinoid therapy, lenalidomide, histone deacetylase inhibitors).

  • Targeted therapy (brentuximab vedotin).

Treatment of fungal mycosis stage III and stage IV (including Sesari syndrome)

 

Treatment for newly diagnosed stage III and IV mycoses, including Sesari syndrome, is palliative (to relieve symptoms and improve quality of life) and may include the following:

  • Psoralen and ultraviolet A radiation therapy (PUVA).

  • Ultraviolet radiation In therapy.

  • Extracorporeal photochemotherapy, administered alone or in combination with total skin electron beam radiation therapy.

  • Radiation therapy with total electron beam therapy of the skin. In some cases, radiation therapy is applied to skin lesions as palliative therapy to reduce tumor size, relieve symptoms, and improve quality of life.

  • Immunotherapy given alone or in combination with skin-targeted therapy.

  • Systemic chemotherapy with one or more drugs that can be combined with skin-directed therapy.

  • Local chemotherapy.

  • Other drug therapy (topical corticosteroids, lenalidomide, bexarotene, histone deacetylase inhibitors).

  • Targeted therapy with brentuximab vedotin.

 

Treatment of recurrent fungal mycosis (including Sesari's syndrome)

 

Treatment for recurrent fungal mycosis, including Sesari's syndrome, can be done in clinical trials and may include the following:

  • Radiation therapy with total electron beam therapy of the skin. In some cases, radiation therapy is applied to skin lesions as palliative therapy to reduce tumor size, relieve symptoms, and improve quality of life.

  • Psoralen and ultraviolet A radiation therapy (PUVA), which can be given in conjunction with immunotherapy.

  • Ultraviolet B radiation.

  • Extracorporeal photochemotherapy.

  • Systemic chemotherapy with one or more drugs.

  • Other drug therapy (topical corticosteroids, retinoid therapy, lenalidomide, histone deacetylase inhibitors).

  • Immunotherapy given alone or in combination with skin-targeted therapy.

  • High-dose chemotherapy and sometimes radiation therapy with stem cell transplantation.

  • Targeted therapy.

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4.4
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4.6.

5. TREATMENT OF PRIMARY CNS LYMPHOMA 

Primary central nervous system (CNS) lymphoma is a disorder in which malignant (cancerous) cells form in the lymphatic tissue of the brain and / or spinal cord.

Lymphoma  is a disease in which malignant (  cancerous)  cells are formed in the lymphatic system. The lymphatic system is part of the immune system and consists of lymph,  lymphatic vessels,  lymph nodes  spleen,  thymus,  tonsils and  bone marrow. Lymphocytes (carried by lymph) travel to and from the central nervous system (CNS). It is believed that some of these lymphocytes become cancerous and cause lymphoma in the central nervous system. Primary CNS lymphoma may begin in the head,  the spinal cord or meninges (the layers that form the outer lining of the brain). Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma).

Якорь 5
Первичная лимфома ЦНС

Anatomy of the lymphatic system showing lymphatic vessels and lymphatic organs including lymph nodes, tonsils, thymus, spleen, and bone marrow. Lymph (clear fluid) and lymphocytes travel through the lymphatic vessels to the lymph nodes, where the lymphocytes destroy harmful substances. Lymph enters the bloodstream through a large vein near the heart.

5.1. Risks of developing CNS lymphoma

A weakened immune system can increase the risk of developing primary CNS lymphoma.

Anything that increases the likelihood of getting sick is called a risk factor. Having a risk factor does not mean that you will get cancer; the absence of risk factors does not mean that you will not get cancer. Talk to your doctor if you think you are at risk.

Primary CNS lymphoma can occur in patients who have acquired immunodeficiency syndrome (AIDS) or other immune system disorders or who have had an organ transplant.  For more information on lymphoma in AIDS patients click here

5.2. Symptoms of Primary CNS Lymphoma  

Signs and symptoms of primary CNS lymphoma may include nausea, vomiting, or seizures.

These and other signs and symptoms can be caused by primary CNS lymphoma or other conditions. Check with your doctor if you have any of the following:

5.3. Tests for the diagnosis of primary CNS lymphoma

Tests that examine the eyes, brain, and spinal cord are used to diagnose primary CNS lymphoma.

 

The following tests and procedures can be used:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness such as bumps or anything else that seems out of the ordinary. A history of the patient's health-related habits as well as past illnesses and treatments will also be recorded.

  • Neurological examination: A series of questions and tests to check the function of the brain, spinal cord and nerves. The exam tests the person's mental state, coordination, ability to walk normally and how well muscles, feelings and reflexes work. It can also be called a neurological or neurological examination.

  • Dilated eye exam: An eye exam in which the pupil is dilated (enlarged) with medicated eye drops so that the doctor can look through the lens and pupil at the retina. The inside of the eye is checked, including the retina and optic nerve. Images can be taken over time to track changes in tumor size. There are several types of eye examinations:

    • Ophthalmoscopy: Examining the inside of the back of the eye to check the retina and optic nerve with a small magnifying lens and light.

    • Slit lamp biomicroscopy: Examining the inside of the eye to check the retina, optic nerve and other parts of the eye using a strong beam of light and a microscope.

  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into the patient's body through a vein. Gadolinium gathers around cancer cells, so they appear brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spine. This is done by inserting a needle between two bones in the spine and into the cerebrospinal fluid and taking a sample of the fluid. A sample of cerebrospinal fluid is checked under a microscope for signs of tumor cells. You can also check the amount of protein and glucose in the sample. Protein levels above normal or below normal glucose may be a sign of a tumor. This procedure is also called LP or lumbar puncture.

  • Stereotactic biopsy: A biopsy procedure that uses a computer and a three-dimensional (3-D) scanning device to locate the tumor and guide tissue removal so that it can be viewed under a microscope to check for signs of cancer.

The following tests can be performed on removed tissue samples:

 

  • Flow cytometry:  a laboratory test that measures the number of cells in a sample, the percentage of living cells in the sample, and certain characteristics of cells such as size, shape, and the presence of tumor (or other) markers on the cell surface. Cells from a sample of blood, bone marrow, or other tissue from a patient are stained with a fluorescent dye, placed in a liquid, and then passed one at a time through a beam of light. The test results are based on how cells stained with a fluorescent dye respond to a beam of light. This test is used to diagnose and treat certain types of cancer, such as leukemia and lymphoma.

  • Immunohistochemistry: A laboratory test that uses antibodies to check for specific antigens (markers) in a tissue sample from a patient. Antibodies are usually associated with an enzyme or fluorescent dye. After antibodies bind to a specific antigen in a tissue sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test is used to diagnose cancer and help distinguish one type of cancer from another.

  • Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a blood or bone marrow sample are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in some chromosomes can be a sign of cancer. Cytogenetic analysis is used to diagnose cancer, plan treatment, or determine the effectiveness of a treatment.

  • FISH (Fluorescence In Situ Hybridization): A laboratory test used to study and count genes or chromosomes in cells and tissues. Pieces of DNA containing fluorescent dyes are made in the laboratory and added to a sample of the patient's cells or tissues. When these stained DNA fragments attach to specific genes or regions of chromosomes in a sample, they light up when viewed under a fluorescent microscope. The FISH test is used to diagnose cancer and plan treatment.

  • Complete blood count (CBC) with differential: A procedure in which a blood sample is taken and checked for the following:

    • The number of red blood cells and platelets.

    • The number and type of leukocytes.

    • The amount of hemoglobin (oxygen-carrying protein) in red blood cells.

    • The portion of a blood sample made up of red blood cells.

 

After primary central nervous system (CNS) lymphoma is diagnosed, tests are done to find out if cancer cells have spread in the brain and spinal cord or into the eyes.

Primary CNS lymphoma usually does not extend beyond the central nervous system or the eye. The process used to determine the spread of cancer is called staging. There is no standardized system for staging primary CNS lymphoma.

The following tests and procedures can be used to plan treatment:

  • CT (computed tomography): a procedure in which a series of detailed pictures of areas inside the body are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to help organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • PET scan (positron emission tomography): a procedure to look for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Malignant tumor cells appear brighter in the image because they are more active and consume more glucose than normal cells. PET scans and CT scans can be performed at the same time. This is called a PET-CT scan.

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • Bone marrow aspiration and biopsy: Removal of bone marrow, blood, and a small piece of bone by inserting a cannula into the hip or sternum. A pathologist examines the bone marrow, blood, and bones under a microscope to look for signs of cancer.

 

Primary CNS lymphoma often recurs (comes back) after treatment.

Primary CNS lymphoma often recurs in the brain, spinal cord, or eyes.

5.4. Overview of treatment options

There are various treatments for patients with primary central nervous system (CNS) lymphoma.

Various treatments are available for patients with primary CNS lymphoma. Some treatments are standard (currently used) and some are in clinical trials. A treatment clinical trial is a scientific study designed to help improve existing treatments or provide information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment can become the standard treatment. Patients may want to take part in a clinical trial. Some clinical trials are only open to patients who have not yet started treatment.

Surgery is not used to treat primary CNS lymphoma.

Four standard treatments are used:

1. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy X-rays or other types of radiation to kill cancer cells or prevent them from growing. External beam radiation therapy uses a device outside the body to direct radiation to an area of the body affected by cancer. Because primary CNS lymphoma spreads throughout the brain, external radiation therapy is applied to the entire brain. This is called whole-brain radiation therapy.

High-dose radiation therapy to the brain can damage healthy tissue and cause disturbances that can affect thinking, learning, problem solving, reading, writing, speaking, and memory. Clinical trials have tested the use of chemotherapy alone or before radiation therapy to reduce the damage to healthy brain tissue that occurs with radiation therapy.

2. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is injected directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ or body cavity such as the abdomen, the drugs mainly target cancer cells in those areas (regional chemotherapy).

How chemotherapy is given depends on where the tumor is in the central nervous system or in the eye. Primary CNS lymphoma can be treated with systemic chemotherapy, intrathecal chemotherapy, and / or intraventricular chemotherapy, in which anticancer drugs are injected into the ventricles (fluid-filled cavities) of the brain. If primary CNS lymphoma is found in the eye, anticancer drugs are given directly.

 

3. Steroid therapy

Steroids are hormones produced naturally by the body. They can also be made in the laboratory and used as medicines. Glucocorticoids are steroid drugs that have anticancer effects for lymphomas.

4. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and target certain cancer cells. Targeted therapy usually does less damage to normal cells than chemotherapy or radiation therapy.

  • Monoclonal antibodies: Monoclonal antibodies are immune system proteins created in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that can promote cancer cell growth. The antibodies are then able to kill cancer cells, block their growth, or prevent them from spreading. Monoclonal antibodies are given by infusion. They can be used alone or to carry drugs, toxins, or radioactive materials directly to cancer cells. Rituximab and nivolumab are types of monoclonal antibodies used to treat newly diagnosed or recurrent primary CNS lymphomas.

  • Tyrosine kinase inhibitors: These small molecule drugs travel across the cell membrane and work inside cancer cells to block signals that cancer cells need to grow and divide. Ibrutinib is a type of tyrosine kinase inhibitor used to treat newly diagnosed or recurrent primary CNS lymphoma.

High-dose chemotherapy with stem cell transplant

High doses of chemotherapy are prescribed to kill cancer cells. Healthy cells, including hematopoietic cells, are also destroyed in cancer treatments. Stem cell transplantation is a treatment to replace hematopoietic cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of a patient or donor, frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and returned to the patient as an infusion. These re-injected stem cells germinate (and regenerate) the body's blood cells.

5.5. Treatment options for primary CNS lymphoma

Treatment of primary CNS lymphoma

 

Treatment for newly diagnosed primary central nervous system (CNS) lymphoma may include the following:

  • Whole Brain Radiation Therapy.

  • Chemotherapy with or without radiation therapy.

  • Targeted therapy alone (rituximab, nivolumab, or ibrutinib).

  • Targeted therapy (rituximab) and chemotherapy.

  • Clinical study on high-dose chemotherapy and stem cell transplantation.

  • A clinical trial of high-dose chemotherapy with or without targeted therapy (rituximab), stem cell transplantation, or whole-brain radiation therapy.

  • Clinical trial of chemotherapy followed by whole-brain radiation therapy or chemotherapy followed by high-dose chemotherapy and stem cell transplantation.

  • Clinical trial of chemotherapy with or without stem cell transplantation.

 

Treatment of primary intraocular lymphoma

Treatment for newly diagnosed primary intraocular lymphoma may include the following:

  • Chemotherapy (intraocular or systemic) with or without whole-brain radiation therapy.

  • Whole Brain Radiation Therapy.

 

Treatment of recurrent primary CNS lymphoma

 

Treatment for recurrent primary central nervous system (CNS) lymphoma may include the following:

  • Chemotherapy.

  • Targeted therapy (rituximab, nivolumab, or ibrutinib).

  • Radiation therapy (if not previously treated).

  • Clinical investigation of a new drug or treatment regimen.

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