Lymphomas are a group of cancers in which cells of the lymphatic system become abnormal and start to grow uncontrollably. Because there is lymph tissue in many parts of the body, lymphomas can start in almost any organ of the body.
The lymph system is made up of ducts or tubules that carry lymph to all parts of the body. Lymph is a milky fluid that contains the lymphocytes or white blood cells. These are the infection-fighting cells of the blood. Small pea-shaped organs are found along the network of lymph vessels. These are called the lymph nodes, and their main function is to make and store the lymphocytes. Clusters of lymph nodes are found in the pelvis region, underarm, neck, chest, and abdomen. The spleen (an organ in the upper abdomen), the tonsils, and the thymus (a small organ found beneath the breastbone) are part of the lymphatic system.
The lymphocyte is the main cell of the lymphoid tissue. There are two main types of lymphocytes: the T lymphocyte and the B lymphocyte. Lymphomas develop from these two cell types. B cell lymphomas are more common among adults, while among children, the incidence of T and B cell lymphomas are almost equal.
The T and the B cell perform different jobs within the immune system. When an infectious bacterium enters the body, the B cell makes proteins called "antibodies." These antibodies attach themselves to the bacteria, and flag them for destruction by other immune cells. The T cells help protect the body against viruses. When a virus enters the cell, it generally produces certain proteins that are projected on the surface of the infected cell. T cells recognize these proteins and produce certain substances (cytokines) that destroy the infected cells. Some of the cytokines made by the T cells attract other cell types, which are capable of digesting the virus-infected cell. The T cells can also destroy some types of cancerous cells.
Lymphomas can be divided into two main types: Hodgkin's lymphoma or Hodgkin's disease, and non-Hodgkin's lymphomas. There are at least 10 types of non-Hodgkin's lymphomas. They are grouped (staged) by how aggressively they grow; slow growing (low grade), intermediate growing, and rapidly growing (high grade); and how far they spread.
A majority of non-Hodgkin's lymphomas begin in the lymph nodes. About 20% start in other organs, such as the lungs, liver or the gastrointestinal tract. Malignant lymphocytes multiply uncontrollably and do not perform their normal functions. Hence, the body's ability to fight infections is affected. In addition, these malignant cells may crowd the bone marrow, and, depending on the stage, prevent the production of normal red blood cells, white blood cells, and platelets. A low red blood cell count causes anemia, while a reduction in the number of platelets makes the person susceptible to excessive bleeding. Cancerous cells can also invade other organs through the circulatory system of the lymph, causing those organs to malfunction.
Causes and symptoms
The exact cause of non-Hodgkin's lymphomas is not known. However, the incidence has increased significantly in the recent years. Part of the increase is due to the AIDS
epidemic. Individuals infected with the AIDS virus have a higher likelihood of developing non-Hodgkin's lymphomas. In general, males are at a higher risk for having non-Hodgkin's lymphomas than are females. The risk increases with age. Though it can strike people as young as 40, people between the ages of 60 and 69 are at the highest risk.
People exposed to certain pesticides and ionizing radiation have a higher than average chance of developing this disease. For example, an increased incidence of lymphomas has been seen in survivors of the atomic bomb explosion in Hiroshima, and in people who have undergone aggressive radiation therapy
. People who suffer from immune-deficient disorders, as well as those who have been treated with immune suppressive drugs for heart or kidney transplants, and for conditions such as rheumatoid arthritis
and autoimmune diseases, are at an increased risk for this disease.
There have been some studies that have shown a loose association between retroviruses, such as HTLV-I, and some rare forms of lymphoma. The Epstein-Barr virus has been linked to Burkitt's lymphoma in African countries. However, a direct cause-and-effect relationship has not been established.
The symptoms of lymphomas are often vague and non-specific. Patients may experience loss of appetite, weight loss, nausea, vomiting, abdominal discomfort, and indigestion
. The patient may complain of a feeling of fullness, which is a result of enlarged lymph nodes in the abdomen. Pressure or pain
in the lower back is another symptom. In the advanced stages, the patient may have bone pain, headaches, constant coughing, and abnormal pressure and congestion in the face, neck, and upper chest. Some may have fevers and night sweats. In most cases, patients go to the doctor because of the presence of swollen glands in the neck, armpits, or groin area. Since all the symptoms are common to many other illnesses, it is essential to seek medical attention if any of the conditions persist for two weeks or more. Only a qualified physician can correctly diagnose if the symptoms are due to lymphoma or some other ailment.
Like all cancers, lymphomas are best treated when found early. However, it is often difficult to diagnose lymphomas. There are no screening tests available, and, since the symptoms are non-specific, lymphomas are rarely recognized in their early stages. Detection often occurs by chance during a routine physical examination
When the doctor suspects lymphoma, a complete medical history is taken, and a thorough physical examination is performed. Enlargement of the lymph nodes, liver, or spleen may suggest lymphomas. Blood tests will determine the cell counts and obtain information on how well the organs, such as the kidney and liver, are functioning.
A biopsy of the enlarged lymph node is the most definitive diagnostic tool for staging purposes. The doctor may perform a bone marrow biopsy. During the biopsy, a cylindrical piece of bone and marrow fluid is removed. They are generally taken out of the hipbone. These samples are sent to the laboratory for examination. In addition to diagnosis, the biopsy may also be repeated during the treatment phase of the disease to see if the lymphoma is responding to therapy.
Once the exact form of lymphoma is known, it is then staged to determine how aggressive it is, and how far it has spread. Staging is necessary to plan appropriate treatment.
Lymphangiograms are x rays of the lymphatic system. In this procedure, a special dye is injected into the lymphatic channels through a small cut (incision) made in each foot. The dye is injected slowly over a period of three to four hours. This dye clearly outlines the lymphatic system and allows it to stand out. Multiple x rays are then taken and any abnormality, if present, is revealed.
Rarely, a lumbar puncture or a spinal tap is performed to check if malignant cells are present in the fluid surrounding the brain. In this test, the physician inserts a needle into the epidural space at the base of the spine and collects a small amount of spinal fluid for microscopic examination.
Treatment options for lymphomas depend on the type of lymphoma and its present stage. In most cases, treatment consists of chemotherapy
, radiotherapy, or a combination of the two methods.
Chemotherapy is the use of anti-cancer drugs to kill cancer
cells. In non-Hodgkin's lymphomas, combination therapy, which involves the use of multiple drugs, has been found more effective than single drug use. The treatment may last about six months, but in some cases may last as long as a year. The drugs may either be administered intravenously (through a vein) in the arm or given orally in the form of pills. If cancer cells have invaded the central nervous system, then chemotherapeutic drugs may be instilled, through a needle in the brain or back, into the fluid that surrounds the brain. This procedure is known as intrathecal chemotherapy.
Radiation therapy, where high-energy ionizing rays are directed at specific portions of the body, such as the upper chest, abdomen, pelvis, or neck, is often used for treatment of lymphomas. External radiation therapy, where the rays are directed from a source outside the body, is the most common mode of radiation treatment.
Bone marrow transplantation
is used in cases where the lymphomas do not respond to conventional therapy, or in cases where the patient has had a relapse or suffers from recurrent lymphomas.
There are two ways of doing bone marrow transplantation. In a procedure called "allogeneic bone marrow transplant," a donor is found whose marrow matches that of the patient. The donor can be a twin (best match), a sibling, or a person who is not related at all. High-dose chemotherapy or radiation therapy is given to eradicate the lymphoma. The donor marrow is then given to replace the marrow destroyed by the therapy.
In "autologous bone marrow transplantation," some of the patient's own bone marrow is harvested, chemically purged, and frozen. High-dose chemotherapy and radiation therapy are given. The marrow that was harvested, purged, and frozen is then thawed and put back into the patient's body to replace the destroyed marrow.
A new treatment option for patients with lymphoma is known as "peripheral stem cell transplantation." In this treatment approach, cells that normally circulate in the blood are collected when the patient has normal blood counts taken, and these cells are saved via a process called "pheresis." Researchers are exploring whether these cells can be used to restore the normal function and development of blood cells, rather than using a bone marrow transplant.
Like all cancers, the prognosis for lymphoma depends on the stage of the cancer, and the patient's age and general health. When all the different types and stages of lymphoma are considered together, only 50% of patients survive 5 years or more after initial diagnosis. This is because some types of lymphoma are more aggressive than others.
The survival rate among children is definitely better than among older people. About 90% of the children diagnosed with early stage disease survive 5 years or more, while only 60-70% of adults diagnosed with low grade lymphomas survive for 5 years or more. The survival rate for children with the more advanced stages is about 75-85%, while among adults it is 40-60%.
Although many cancers may be prevented by making diet and life style changes which reduce risk factors, there is currently no known way to prevent lymphomas. Protecting oneself from developing AIDS, which may be a risk factor for lymphomas, is the only preventive measure that can be practiced.
At present, there are no special tests that are available for early detection of non-Hodgkin's lymphomas. Paying prompt attention to the signs and symptoms of this disease, and seeing a doctor if the symptoms persist, are the best strategies for an early diagnosis of lymphoma. Early detection affords the best chance for a cure.
— Proteins made by the B lymphocytes in response to the presence of infectious agents such as bacteria or viruses in the body.
— The surgical removal and microscopic examination of living tissue for diagnostic purposes.
Growth factors (cytokines)
— Chemicals made by the cells that act on other cells to stimulate or inhibit their function. Cytokines that stimulate growth are called "growth factors."
American Cancer Society. 1599 Clifton Rd., NE, Atlanta, GA 30329-4251. (800) 227-2345. http://www.cancer.org.
Cancer Research Institute. 681 Fifth Ave., New York, N.Y. 10022. (800) 992-2623. http://www.cancerresearch.org.
Leukemia Society of America, Inc. 600 Third Ave., New York, NY 10016. (800) 955 4572. http://www.leukemia.org.
Lymphoma Research Foundation. 8800 Venice Boulevard, Suite 207, Los Angeles, CA 90034. (310) 204 7040.
National Cancer Institute. Building 31, Room 10A31, 31 Center Drive, MSC 2580, Bethesda, MD 20892-2580. (800) 422-6237. http://www.nci.nih.gov.
Oncolink. University of Pennsylvania Cancer Center. http://cancer.med.upenn.edu.
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