AIDS-Related Lymphoma
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 diseases that invade the body. People with HIV disease
have an increased risk of developing infections, lymphoma, and 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.
Lymphomas are cancers that affect the white blood cells of the
lymph system, part of the body's immune system. The lymph system is made up of
the following:
- Lymph: Colorless, watery fluid that travels through the
lymph system and carries white blood cells called lymphocytes. Lymphocytes
protect the body against infections and the growth of tumors.
- Lymph 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 infection and disease.
Lymph nodes are located along the network of lymph vessels found throughout
the body. Clusters of lymph nodes are found in the underarm, pelvis, neck,
abdomen, and groin.
- Spleen: An organ that makes lymphocytes, filters the
blood, stores blood cells, and destroys old blood cells. The spleen is on
the left side of the abdomen near the stomach.
- Thymus: An organ in which lymphocytes grow and multiply.
The thymus is in the chest behind the breastbone.
- Tonsils: Two small masses of lymph tissue at the back of
the throat. The tonsils make lymphocytes.
- Bone marrow: The soft, spongy tissue in the center of
large bones. Bone marrow makes white blood cells, red blood cells, and
platelets.
There are many different types of lymphoma.
Lymphomas are divided into two general types: Hodgkin lymphoma
and non-Hodgkin lymphoma. Both Hodgkin lymphoma and non-Hodgkin lymphoma may
occur in AIDS patients, but non-Hodgkin lymphoma is more common. When a person
with AIDS has non-Hodgkin lymphoma, it is called an AIDS-related 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 lymphoma is usually aggressive. There are three
main types of AIDS-related lymphoma:
- Diffuse large B-cell lymphoma
- B-cell immunoblastic lymphoma
- Small non- cleaved cell lymphoma
Possible signs of AIDS-related lymphoma include weight loss,
fever, and night sweats.
These and other symptoms may be caused by AIDS-related lymphoma.
Other conditions may cause the same symptoms. A doctor should be consulted if
any of the following problems occur:
- Weight loss or fever for no known reason.
- Night sweats
- Painless, swollen lymph nodes in the neck, chest, underarm, or groin.
- A feeling of fullness below the ribs.
Tests that examine the body and lymph system are used to
help detect (find) and diagnose AIDS-related lymphoma.
The following tests and procedures may be used:
Physical exam and history: An exam of the body to check general
signs of health, including checking for signs of disease, such as lumps or
anything else that seems unusual. A history of the patient’s health habits and
past illnesses and treatments will also be taken.
Complete blood count (CBC): A procedure in which a sample of
blood is drawn and checked for the following:
- The number of red blood cells, white blood cells, and platelets.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the sample made up of red blood cells.
Lymph node biopsy: The removal of all or part of a lymph
node. A pathologist views the tissue under a microscope to look for cancer
cells. One of the following types of biopsies may be done:
- Excisional biopsy: The removal of an entire lymph node.
- Incisional biopsy: The removal of part of a lymph node.
- Core biopsy: The removal of tissue from a lymph node using a
wide needle.
- Fine-needle aspiration (FNA) biopsy: The removal of tissue
from a lymph node using a thin needle.
Bone marrow aspiration and biopsy: The removal of bone
marrow, blood, and a small piece of bone by inserting a hollow needle into the
hipbone or breastbone. A pathologist views the bone marrow, blood, and bone
under a microscope to look for abnormal cells.
HIV test: A test to measure the level of HIV antibodies in a
sample of blood. Antibodies are made by the body when it is invaded by a foreign
substance. A high level of HIV antibodies may mean the body has been infected with HIV.
Epstein-Barr virus (EBV) test: A test to measure the level
of EBV antibodies in a sample of blood, tissue, or cerebrospinal fluid (CSF).
Antibodies are made by the body when it is invaded by a foreign substance. A
high level of EBV antibodies may mean the body has been infected with EBV.
Chest x-ray: An x-ray of the organs and bones inside the
chest. An x-ray is a type of energy beam that can go through the body and onto
film, making a picture of areas inside the body.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- The stage of the cancer.
- The number of CD4 lymphocytes (a type of white blood cell) in the blood.
- Whether the patient has ever had AIDS-related infections.
- The patient's ability to carry out regular daily activities.
Stages of AIDS-Related Lymphoma
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:
CT scan (CAT scan): A procedure that makes a series of
detailed pictures of areas inside the body, taken from different angles. The
pictures are made by a computer linked to an x-ray machine. A dye may be
injected into a vein or swallowed to help the organs or tissues show up more
clearly. This procedure is also called computed tomography, computerized
tomography, or computerized axial tomography.
PET scan (positron emission tomography scan): A procedure to
find 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
makes a picture of where glucose is being used in the body. Malignant tumor
cells show up brighter in the picture because they are more active and take up
more glucose than normal cells do.
MRI (magnetic resonance imaging): A procedure that uses a
magnet, radio waves, and a computer to make a series of detailed pictures of
areas inside the body. A substance called gadolinium is injected into the
patient through a vein. The gadolinium collects around the cancer cells so they
show up brighter in the picture. This procedure is also called nuclear magnetic
resonance imaging (NMRI).
Bone marrow aspiration and biopsy: The removal of bone
marrow, blood, and a small piece of bone by inserting a hollow needle into the
hipbone or breastbone. A pathologist views the bone marrow, blood, and bone
under a microscope to look for abnormal cells.
Lumbar puncture: A procedure used to collect cerebrospinal
fluid from the spinal column. This is done by placing a needle into the spinal
column. This procedure is also called an LP or spinal tap.
Blood chemistry studies: A procedure in which a blood sample
is checked to measure the amounts of certain substances released into the blood
by organs and tissues in the body. An unusual (higher or lower than normal)
amount of a substance can be a sign of disease in the organ or tissue that makes
it. The blood sample will be checked for the level of LDH (lactate
dehydrogenase).
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system
and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries
and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor
and travel through the lymph or blood to other places in the body, another
(secondary) tumor may form. This process is called metastasis. The secondary
(metastatic) tumor is the same type of cancer as the primary tumor. For example,
if breast cancer spreads to the bones, the cancer cells in the bones are
actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
Stages of AIDS-related lymphoma may include E and S.
AIDS-related lymphoma may be described as follows:
- E: "E" stands for extranodal and means the cancer is found in an
area or organ other than the lymph nodes or has spread to tissues beyond, but
near, the major lymphatic areas.
- S: "S" stands for spleen and means the cancer is found in the
spleen.
The following stages are used for AIDS-related lymphoma:
Stage I
Stage I AIDS-related lymphoma is divided into stage I and stage IE.
- Stage I: Cancer is found in one lymph node group.
- Stage IE: Cancer is found in an area or organ other than the
lymph nodes.
Stage II
Stage II AIDS-related lymphoma is divided into stage II and stage IIE.
- Stage II: Cancer is found in two or more lymph node groups on
the same side of the diaphragm (the thin muscle below the lungs that helps
breathing and separates the chest from the abdomen).
- Stage IIE: Cancer is found in an area or organ other than the
lymph nodes and in lymph nodes near that area or organ, and may have spread to
other lymph node groups on the same side of the diaphragm.
Stage III
Stage III AIDS-related lymphoma is divided into stage III, stage IIIE, stage IIIS, and stage IIIS+E.
- Stage III: Cancer is found in lymph node groups on both
sides of the diaphragm (the thin muscle below the lungs that helps breathing and
separates the chest from the abdomen).
- Stage IIIE: Cancer is found in lymph node groups on both
sides of the diaphragm and in an area or organ other than the lymph nodes.
- Stage IIIS: Cancer is found in lymph node groups on both
sides of the diaphragm and in the spleen.
- Stage IIIS+E: Cancer is found in lymph node groups on both
sides of the diaphragm, in an area or organ other than the lymph nodes, and in the spleen.
Stage IV
In stage IV AIDS-related lymphoma, the cancer either:
- is found throughout one or more organs other than the lymph
nodes and may be in lymph nodes near those organs; or
- is found in one organ other than the lymph nodes and has spread
to lymph nodes far away from that organ.
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:
Peripheral/systemic lymphoma
Lymphoma that starts in lymph nodes or other organs of the lymph
system is called peripheral/systemic lymphoma. The lymphoma may spread
throughout the body, including to the brain or bone marrow.
Primary CNS lymphoma
Primary CNS lymphoma starts in the central nervous system (brain
and spinal cord). Lymphoma that starts somewhere else in the body and spreads to
the central nervous system is not primary CNS lymphoma.
Treatment Option Overview
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 further damage.
For this reason, patients who have AIDS-related lymphoma are usually treated with lower doses
of drugs than lymphoma patients who do not have AIDS.
Highly-active antiretroviral therapy (HAART) is used to slow
progression of HIV (which is a retrovirus). Treatment with HAART may allow some
patients to safely receive anticancer drugs in standard or higher doses.
Medicine to prevent and treat infections, which can be serious, is also used.
AIDS-related lymphoma usually grows faster than lymphoma that is
not AIDS-related and it is more likely to spread to other parts of the body. In
general, AIDS-related lymphoma is harder to treat.
Three types of standard treatment are used
Chemotherapy
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 spinal
column (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 the type and stage
of the cancer being treated.
Intrathecal chemotherapy may be used in patients who are more
likely to have lymphoma in the central nervous system (CNS).
Colony-stimulating factors are sometimes given together with chemotherapy. This helps
lessen the side effects chemotherapy may have on the bone marrow.
Radiation therapy
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. External radiation therapy
uses a machine outside the body to send radiation toward the cancer. Internal
radiation therapy uses a radioactive substance sealed in needles, seeds, wires,
or catheters that are placed directly into or near the cancer. The way the
radiation therapy is given depends on the type and stage of the cancer being treated.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a method 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.
New types of treatment are being tested in clinical trials.
Below describes treatments that are being studied in clinical trials. It may not mention
every new treatment being studied.
Targeted therapy
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 one type of targeted therapy being studied
in the treatment of AIDS-related lymphoma.
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.
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.
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.
This is sometimes called re-staging.
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.
Treatment Options for Aids-Related Lymphoma
For some types or stages of cancer, there may not be any trials
listed. Check with your doctor for clinical trials that are not listed here but
may be right for you.
AIDS-Related Peripheral/Systemic Lymphoma
There is no standard treatment plan for AIDS-related
peripheral/systemic lymphoma. Treatment is adjusted for each patient and is
usually one or more of the following:
- Combination chemotherapy.
- High-dose chemotherapy and stem cell transplant.
- A clinical trial of monoclonal antibodies.
- A clinical trial of different treatment combinations.
AIDS-Related Primary Central Nervous System Lymphoma
Treatment of AIDS-related primary central nervous system
lymphoma is usually radiation therapy.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical
Trials Registry 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.
For more information from the NCI, please write to this address:
NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322
U.S. residents may call the National Cancer Institute's (NCI's) Cancer
Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through
Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY
equipment may call 1-800-332-8615. Information about ongoing clinical trials is
available from the NCI Web site.
Source: National Institutes of Health; National Cancer Institute
index#6153
| 
