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Because lymph tissue is found throughout the body, adult non-Hodgkin lymphoma can begin in almost any part of the body. Cancer can spread to the liver and many other organs and tissues. Non-Hodgkin lymphoma in pregnant women is the same as the disease in non-pregnant women of childbearing age. However, treatment is different for pregnant women. Non-Hodgkin lymphoma can occur in both adults and children. Treatment for children, however, is different than treatment for adults.
Waldenström macroglobulinemia is a type of non-Hodgkin lymphoma.
Waldenström macroglobulinemia begins in a type of white blood cell called B lymphocytes. Certain B lymphocytes multiply out of control and make large amounts of a protein called monoclonal immunoglobulin M (IgM) antibody. High levels of IgM in the blood cause the blood to thicken and leads to many of the symptoms of Waldenström macroglobulinemia. Waldenström macroglobulinemia is also called lymphoplasmacytic lymphoma.
Age, gender, and a weakened immune system can affect the risk of adult non-Hodgkin lymphoma.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult non-Hodgkin lymphoma include the following:
- Being older, male, or white.
- Having one of the following medical conditions:
- An inherited immune disorder (for example, hypogammaglobulinemia or Wiskott-Aldrich syndrome).
- An autoimmune disease (for example, rheumatoid arthritis, psoriasis, or Sjögren syndrome).
- HIV /AIDS.
- Human T-lymphotrophic virus type I or Epstein-Barr virus.
- A history of Helicobacter pylori infection.
- Taking immunosuppressant drugs after an organ transplant.
- Being exposed to certain pesticides.
- A diet high in meats and fat.
- Past treatment for Hodgkin lymphoma.
Signs and symptoms of adult non-Hodgkin lymphoma include fever, sweating, weight loss, and fatigue.
These and other signs and symptoms may be caused by adult non-Hodgkin lymphoma or by other conditions. Check with your doctor if you have any of the following:
- Painless swelling in the lymph nodes in the neck, underarm, groin, or stomach
- Fever for no known reason
- Drenching night sweats
- Feeling very tired
- Weight loss for no known reason
- Skin rash or itchy skin
- Pain in the chest, abdomen, or bones for no known reason
Signs and symptoms of Waldenström macroglobulinemia depend on the part of the body affected. Most patients with Waldenström macroglobulinemia have no signs or symptoms. Check with your doctor if you have any of the following:
- Feeling very tired
- Easy bruising or bleeding, such as nosebleeds or bleeding from the gums
- Vision changes, such as blurred vision or blind spots
- Pain, tingling, or numbness, especially in the hands, feet, fingers, or toes
- Pain or a feeling of fullness below the ribs on the left side
- Painless lumps in the neck, underarm, stomach, or groin
- Weight loss for no known reason
Tests that examine the body and lymph system are used to help detect (find) and diagnose adult non-Hodgkin 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.
- Blood and urine immunoglobulin studies: A procedure in which a blood or urine sample is checked to measure the amounts of certain antibodies (immunoglobulins). In Waldenström macroglobulinemia, immunoglobulin M (IgM) and beta-2-microglobulin is measured. A higher- or lower-than-normal amount of these substances can be a sign of disease.
- Blood viscosity test: A procedure in which a blood sample is checked to see how “thick” the blood is. In Waldenström macroglobulinemia, when the amount of monoclonal immunoglobulin M (IgM) antibody in the blood becomes very high, the blood thickens and may cause signs or symptoms.
- Flow cytometry: A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. This test is used to diagnose Waldenström macroglobulinemia.
- Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
- 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. A pathologist views the cerebrospinal fluid under a microscope to look for signs of cancer.
- 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 part of a lymph node using a wide needle.
- Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.
- Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and alaparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.
- Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.
If cancer is found, the following tests may be done to study the cancer cells:
- Immunohistochemistry: A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.
- Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
- Immunophenotyping: A process used to identify cells, based on the types of antigens ormarkers on the surface of the cell. This process is used to diagnose specific types of leukemiaand lymphoma by comparing the cancer cells to normal cells of the immune system.
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 type of non-Hodgkin lymphoma
- The amount of lactate dehydrogenase (LDH) in the blood
- The amount of beta-2-microglobulin in the blood (for Waldenström macroglobulinemia)
- The patient’s age and general health
- Whether the lymphoma has just been diagnosed or has recurred (come back)
For non-Hodgkin lymphoma during pregnancy, the treatment options also depend on:
- The wishes of the patient
- Which trimester of pregnancy the patient is in
Some types of non-Hodgkin lymphoma spread more quickly than others do. Most non-Hodgkin lymphomas that occur during pregnancy are aggressive. Delaying treatment of aggressive lymphoma until after the baby is born may lessen the mother’s chance of survival. Immediate treatment is often recommended, even during pregnancy.
After adult non-Hodgkin 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 the type of cancer and 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 of the disease in order to plan treatment. The following tests and procedures may be used in the staging process:
- The number of red blood cells and platelets
- The number and type of white blood cells
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells
- The portion of the blood sample made up of red blood cells
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.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lung, lymph nodes, and liver, 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 scannerrotates 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. 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 needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
For pregnant women with non-Hodgkin lymphoma, staging tests that protect the fetus from the harms of radiation are used. These include MRI, bone marrow aspiration and biopsy, lumbar puncture, andultrasound, which do not use radiation. An ultrasound exam is a procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood:
- Tissue. The cancer spreads from where it began by growing into nearby areas.
- Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
- Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Stages of adult non-Hodgkin lymphoma may include E and S.
Adult non-Hodgkin lymphoma may be described as follows:
- E: “E” stands for extranodal and means the cancer is found in an area or organ other than thelymph 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.
Adult non-Hodgkin lymphomas are also described based on how fast they grow and where the affected lymph nodes are in the body.
Indolent or aggressive:
- Indolent lymphomas: These tend to grow and spread slowly and have few symptoms.
- Aggressive lymphomas: These grow and spread quickly and have severe symptoms.Lymphoblastic lymphoma, diffuse small noncleaved cell lymphoma /Burkitt lymphoma, andmantle cell lymphoma are three types of aggressive adult non-Hodgkin lymphoma. Aggressive lymphomas are seen more often in patients who are HIV -positive (AIDS -related lymphoma).
Contiguous or non-contiguous:
- Contiguous lymphomas: Lymphomas in which the lymph nodes with cancer are next to each other.
- Non-contiguous lymphomas: Lymphomas in which the lymph nodes with cancer are not next to each other, but are on the same side of the diaphragm.
There is no standard staging system for Waldenström macroglobulinemia.
There are different types of treatment for patients with non-Hodgkin lymphoma.
Different types of treatment are available for patients with non-Hodgkin 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.
For pregnant women with non-Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mother’s wishes, the stage of the non-Hodgkin lymphoma, and the age of the fetus. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
Six types of standard treatment are used:
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells and keep them from growing. There are two types of radiation therapy. External radiationtherapy uses a machine outside the body to send radiation toward the cancer. Internal radiationtherapy 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.
For pregnant women with non-Hodgkin lymphoma, radiation therapy should be given after delivery, if possible, to avoid any risk to the fetus. If treatment is needed right away, pregnant women may decide to continue the pregnancy and receive radiation therapy. However, lead used to shield the fetus may not protect it from scattered radiation that could possibly cause cancer in the future.
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 avein 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, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). To treat certain types of adult non-Hodgkin lymphoma that spread to the brain, CNS prophylaxis (chemotherapy given to kill cancer cells in the brain or spinal cord) may be used. The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Combination chemotherapy is treatment using two or more anticancer drugs. Steroid drugs may be added, to relieve swelling and inflammation.
In pregnant women, the fetus is exposed to chemotherapy when the mother is treated, and some anticancer drugs cause birth defects. Because anticancer drugs are passed to the fetus through the mother, both must be watched closely when chemotherapy is given.
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 and proteasome inhibitor therapy are two types of targeted therapy used to treat adult non-Hodgkin 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 byinfusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies that have been joined to radioactive material are called radiolabeledmonoclonal antibodies.
Proteasome inhibitor therapy blocks the action of proteasomes in cancer cells and may prevent the growth of tumors.
If the blood becomes thick with extra antibody proteins and affects circulation, plasmapheresis is done to remove extra plasma and antibody proteins from the blood. In this procedure, blood is removed from the patient and sent through a machine that separates the plasma (the liquid part of the blood) from the blood cells. The patient’s plasma contains the unneeded antibodies and is not returned to the patient. The normal blood cells are returned to the bloodstream along with donated plasma or a plasma replacement. Plasmapheresis does not keep new antibodies from forming.
Biologic therapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
Interferon is a type of biologic therapy. It affects the division of cancer cells and can slow tumor growth.
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change.
Vaccine therapy is a type of biologic therapy. Biologic therapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Vaccine therapy can also be a type of targeted therapy.
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 re-infused stem cells grow into (and restore) the body’s blood cells.