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AIDS-related lymphoma is a disease in which malignant (cancer) cells form in the lymph system of patients who have acquired immunodeficiency syndrome (AIDS).
AIDS is caused by the human immunodeficiency virus (HIV), which attacks and weakens the body's immune system. The immune system is then unable to fight infection and disease. People with HIV disease have an increased risk of infection and lymphoma or other types of cancer. A person with HIV disease who develops certain types of infections or cancer is then diagnosed with AIDS. Sometimes, people are diagnosed with AIDS and AIDS-related lymphoma at the same time. For information about AIDS and its treatment, please see the AIDSinfo website.
AIDS-related lymphoma is a type of cancer that affects the lymph system, which is part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system is made up of the following:
Lymph tissue is also found in other parts of the body such as the brain, stomach, thyroid gland, and skin.
Sometimes AIDS-related lymphoma occurs outside the lymph nodes in the bone marrow, liver, meninges (thin membranes that cover the brain) and gastrointestinal tract. Less often, it may occur in the anus, heart, bile duct, gingiva, and muscles.
Anatomy of the lymph system, showing the lymph vessels and lymph organs including lymph nodes, tonsils, thymus, spleen, and bone marrow. Lymph (clear fluid) and lymphocytes travel through the lymph vessels and into the lymph nodes where the lymphocytes destroy harmful substances. The lymph enters the blood through a large vein near the heart.
There are many different types of lymphoma.
Lymphomas are divided into two general types:
Both Hodgkin lymphoma and non-Hodgkin lymphoma may occur in patients with AIDS, but non-Hodgkin lymphoma is more common. When a person with AIDS has non-Hodgkin lymphoma, it is called AIDS-related lymphoma. When AIDS-related lymphoma occurs in the central nervous system (CNS), it is called AIDS-related primary CNS lymphoma.
Non-Hodgkin lymphomas are grouped by the way their cells look under a microscope. They may be indolent (slow-growing) or aggressive (fast-growing). AIDS-related lymphomas are aggressive. There are two main types of AIDS-related non-Hodgkin lymphoma:
For more information about lymphoma or AIDS-related cancers, see the following PDQ summaries:
Signs of AIDS-related lymphoma include weight loss, fever, and night sweats.
These and other signs and symptoms may be caused by AIDS-related lymphoma or by other conditions. Check with your doctor if you have any of the following:
Tests that examine the lymph system and other parts of the body are used to help detect (find) and diagnose AIDS-related lymphoma.
The following tests and procedures may be used:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
After AIDS-related lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.
The process used to find out if cancer cells have spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment, but AIDS-related lymphoma is usually advanced when it is diagnosed.
The following tests and procedures may be used in the staging process:
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood:
Stages of AIDS-related lymphoma may include E and S.
AIDS-related lymphoma may be described as follows:
The following stages are used for AIDS-related lymphoma:
Stage I AIDS-related lymphoma. Cancer is found in one lymphatic area (lymph nodes, tonsils, thymus, or spleen). In stage IE (not shown), cancer is found in one organ or area outside the lymph nodes.
Stage I AIDS-related lymphoma is divided into stage I and stage IE.
Stage II AIDS-related lymphoma is divided into stage II and stage IIE.
Stage III AIDS-related lymphoma. Cancer is found in one or more lymph node groups above and below the diaphragm (a). In stage IIIE, cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area (b). In stage IIIS, cancer is found in lymph node groups above and below the diaphragm (a) and in the spleen (c). In stage IIIE plus S, cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area (b), and in the spleen (c).
Stage III AIDS-related lymphoma is divided into stage III, stage IIIE, stage IIIS, and stage IIIE+S.
Stage IV AIDS-related lymphoma. Cancer is found throughout one or more organs that are not part of a lymphatic area (lymph nodes, tonsils, thymus, or spleen) (a); or in one organ that is not part of a lymphatic area and has spread to lymph nodes far away from that organ (b); or cerebrospinal fluid (not shown), the liver, bone marrow, or lungs.
In stage IV AIDS-related lymphoma, the cancer:
Patients who are infected with the Epstein-Barr virus or whose AIDS-related lymphoma affects the bone marrow have an increased risk of the cancer spreading to the central nervous system (CNS).
For treatment, AIDS-related lymphomas are grouped based on where they started in the body, as follows:
Lymphoma that starts in the lymph system or elsewhere in the body, other than the brain, is called peripheral/systemic lymphoma. It may spread throughout the body, including to the brain or bone marrow. It is often diagnosed in an advanced stage.
Primary CNS lymphoma
Primary CNS lymphoma starts in the central nervous system (brain and spinal cord). It is linked to the Epstein-Barr virus. Lymphoma that starts somewhere else in the body and spreads to the central nervous system is not primary CNS lymphoma.
There are different types of treatment for patients with AIDS-related lymphoma.
Different types of treatment are available for patients with AIDS-related lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Treatment of AIDS-related lymphoma combines treatment of the lymphoma with treatment for AIDS.
Patients with AIDS have weakened immune systems and treatment can cause the immune system to become even weaker. For this reason, treating patients who have AIDS-related lymphoma is difficult and some patients may be treated with lower doses of drugs than lymphoma patients who do not have AIDS.
Combined antiretroviral therapy (cART) is used to lessen the damage to the immune system caused by HIV. Treatment with combined antiretroviral therapy may allow some patients with AIDS-related lymphoma to safely receive anticancer drugs in standard or higher doses. In these patients, treatment may work as well as it does in lymphoma patients who do not have AIDS. Medicine to prevent and treat infections, which can be serious, is also used.
For more information about AIDS and its treatment, please see the AIDSinfo website.
Four types of standard treatment are used:
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by 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 placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug.
The way the chemotherapy is given depends on where the cancer has formed. Intrathecal chemotherapy may be used in patients who are more likely to have lymphoma in the central nervous system (CNS).Intrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed.
Chemotherapy is used in the treatment of AIDS-related peripheral/systemic lymphoma. It is not yet known whether it is best to give combined antiretroviral therapy at the same time as chemotherapy or after chemotherapy ends.
Colony-stimulating factors are sometimes given together with chemotherapy. This helps lessen the side effects chemotherapy may have on the bone marrow.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:
The way the radiation therapy is given depends on where the cancer has formed. External radiation therapy is used to treat AIDS-related primary CNS lymphoma.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
Stem cell transplant. (Step 1): Blood is taken from a vein in the arm of the donor. The patient or another person may be the donor. The blood flows through a machine that removes the stem cells. Then the blood is returned to the donor through a vein in the other arm. (Step 2): The patient receives chemotherapy to kill blood-forming cells. The patient may receive radiation therapy (not shown). (Step 3): The patient receives stem cells through a catheter placed into a blood vessel in the chest.
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy.
Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. These may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Rituximab is used in the treatment of AIDS-related peripheral/systemic lymphoma.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
AIDS-Related Peripheral/Systemic Lymphoma
Treatment of AIDS-related peripheral/systemic lymphoma may include the following:
Check the list of NCI-supported cancer clinical trials that are now accepting patients with AIDS-related peripheral/systemic lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
AIDS-Related Primary Central Nervous System Lymphoma
Treatment of AIDS-related primary central nervous system lymphoma may include the following:
Check the list of NCI-supported cancer clinical trials that are now accepting patients with AIDS-related primary CNS lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
For more information from the National Cancer Institute about AIDS-related lymphoma, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
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This PDQ cancer information summary has current information about the treatment of AIDS-related lymphoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
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Clinical Trial Information
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Clinical trials are listed in PDQ and can be found online at NCI's website. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).
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PDQ® Adult Treatment Editorial Board. PDQ AIDS-Related Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/aids-related-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389358]
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Last Revised: 2016-10-12
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Last modified on: 8 September 2017