$Unique_ID{BRK03932} $Pretitle{} $Title{Leukemia, Chronic Lymphocytic} $Subject{Leukemia Chronic Lymphocytic Chronic Lymphatic Leukemia CLL Hairy Cell Leukemia Hodgkin's Disease Non-Hodgkin's Lymphoma Waldenstrom Macroglobulinemia } $Volume{} $Log{} Copyright (C) 1991, 1992 National Organization for Rare Disorders, Inc. 858: Leukemia, Chronic Lymphocytic ** IMPORTANT ** It is possible the main title of the article (Chronic Lymphocytic Leukemia) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names and disorder subdivisions covered by this article. Synonyms Chronic Lymphatic Leukemia CLL Information on the following diseases can be found in the Related Disorders section of this report: Hairy Cell Leukemia Hodgkin's Disease Non-Hodgkin's Lymphoma Waldenstrom Macroglobulinemia General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the Resources section of this report. Chronic Lymphocytic Leukemia is a malignant blood disorder in which there is an increased number of white blood cells formed in the lymphoid tissue. This uncontrolled buildup and enlargement of lymphoid tissue can occur in various sites of the body such as the lymph nodes, spleen, bone marrow, and lungs. There are many different forms of Leukemia which are all characterized by an overabundance of white blood cells. In Chronic Lymphocytic Leukemia the disease occurs in the lymphoid tissue. The lymph vessels, which return fluids to the circulatory system, and the lymph nodes, which are a mass of tissue separated into compartments by connective tissue, make up the immune system. The lymph nodes serve as filters, removing foreign particles, tissue debris, and bacterial cells from the circulation. When this system is not working properly, the body's defenses cannot fight off foreign particles. In the majority of cases, Chronic Lymphocytic Leukemia is the result of a rapid production of B lymphocyte cells (a short-lived type of white blood cell that is responsible for the production of vertebrate serum proteins that include antibodies). A small percentage of Chronic Lymphocytic Leukemia cases stem from the overproduction of T lymphocyte cells (a type of white blood cell that have a long life and are important in the resistance of disease). Symptoms One quarter of the patients with Chronic Lymphocytic Leukemia have no symptoms when first diagnosed. The disease is discovered during a routine exam or blood test. Early signs of Chronic Lymphocytic Leukemia may be fatigue, weight loss, loss of appetite (anorexia), labored breathing, low-grade fever, a feeling of fullness in the abdomen due to an enlarged spleen, and night sweats. Bacterial infections such as skin infections, fluid and inflammation of the lungs (pneumonia), and inflammation of the sinuses (sinusitis) often occur. As the disorder advances, the patient loses the ability to fight off infections. Viral infections become an increasing concern. An abnormally high sensitivity to insect bites may also occur. In the later stages of the disorder, the liver, spleen, and lymph nodes may steadily increase in size. Chronic Lymphocytic Leukemia may also invade other tissues such as the skin, eye socket (orbit), mucous membrane that lines the inside of the eyelids (conjunctivae), lungs, sacs that line the chest (pleura), heart, and gastrointestinal tract. Swelling and a yellow pigment of the skin (jaundice) may also occur. Causes Like most other forms of leukemia, the exact cause of Chronic Lymphocytic Leukemia is not known. When the disorder results from a rapid production of B Lymphocyte cells, it is often inherited through autosomal dominant genes. (Human traits including the classic genetic diseases, are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In dominant disorders, a single copy of the disease gene (received from either the mother or father) will be expressed "dominating" the normal gene and resulting in appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50% for each pregnancy regardless of the sex of the resulting child.) Chronic Lymphocytic Leukemia occurs in multiple family members more often than any other type of leukemia. Sibling, especially brothers, seem to have the highest occurrence. Other types of leukemia can occur from exposure to radiation. However, the cause is unknown in most cases. Affected Population Chronic Lymphocytic Leukemia is twice as common in males as in females. This is the most common type of leukemia occuring in multiple family members. The average age of patients is sixty, and the occurrence increases with age. Chronic Lymphocytic Leukemia almost never affects children and is rare under the age of thirty. In the United States, three out of every 100,000 people will be afflicted with this disorder. Related Disorders Symptoms of the following disorders can be similar to those of Chronic Neutropenia. Comparisons may be useful for a differential diagnosis. Hairy Cell Leukemia is a type of blood cancer characterized by the presence of abnormal mononuclear blood cells called "hairy cells," and by a deficiency of other blood cell elements. Symptoms of this disorder may include fatigue, weight loss, abdominal pain, weakness, and easy bruising. (For more information on this disorder, choose "Hairy Cell Leukemia" as your search term in the Rare Disease Database. Hodgkin's Disease is a form of cancer of the lymphatic system, especially the lymph nodes (places where lymphatic vessels unite). Fever, night sweats, swollen glands, and weight loss may occur. Most often a lymph node in the neck is affected. (For more information on this disorder, choose "Hodgkin's Disease" as your search term in the Rare Disease Database). Infectious Mononucleosis is a very prevalent disorder characterized by fever, fatigue, swollen glands, and an abnormally large number of lymphocytes (type of white blood cells) in the blood. It is caused by the Epstein-Barr virus. This disorder is most prevalent in places where young people live in close quarters such as colleges or military. Non-Hodgkin's Lymphomas are a group of cancers of the lymphatic system. Swollen lymph nodes in the neck or groin occur and usually spread throughout the body. Symptoms of this disorder may be anemia (abnormally low levels of red blood cells), weight loss, fever, night sweats, and weakness. (For more information on this disorder, choose "Non-Hodgkin's Lymphoma" as your search term in the Rare Disease Database). Waldenstrom's Macroglobulinemia is a malignant lymph node and blood cell disorder. Large quantities of homogenous immunoglobulin-M (IgM) protein molecules are present in the blood. The most frequent symptoms are an enlarged spleen and liver, abnormalities of the peripheral lymph glands, weakness, anemia, fatigue, and excessive bleeding, especially from the nose and mouth. This disorder tends to run in families and occurs mainly in older males. (For more information on this disorder, choose "Waldenstrom" as your search term in the Rare Disease Database). Therapies: Standard Treatment of Chronic Lymphocytic Leukemia includes platelet transfusions which are used for bleeding associated with a persistent decrease in the number blood platelets (thrombocytopenia). When anemia is present, transfusions of packed red blood cells are usually given. Antibiotics are used to combat bacterial infections usually related to a decrease in the number of leukocytes (lymphopenia) and a low level of gammaglobulin in the blood. Radiation, anticancer, and corticosteroid drugs may be used to prevent the spread of white cells in the lymph tissues. Genetic counseling may be of benefit for patients and families if they have the hereditary form of this disorder. Therapies: Investigational Various antibodies (MoAbs) derived from a single cell are being used in large quantities against the spreading of Chronic Lymphocytic Leukemia. MoAbs combined with other drugs are also being tested at this time. The orphan drug Marinol is currently under investigation as a treatment for Chronic Lymphocytic Leukemia. The drug is manufactured by Warner Lambert Company, 2800 Plymouth Rd., Ann Arbor, MI, 48105-2430. The orphan product Technetium Tc-99m Murine Monoclonal Antibody (IgG2a) to BCE (Immuraid-LL-2(99mTc) is being investigated for use in evaluating the extent of disease in patients with Chronic Lymphocytic Leukemia and some forms of non-Hodgkin's type Lymphoma. The product is sponsored by Immunomedics, Inc., 150 Mt. Bethel Rd., Warren, NJ, 07059. This disease entry is based upon medical information available through September 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Chronic Lymphocytic Leukemia, please contact: National Organization for Rare Disorders P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Leukemia Society of America 733 Third Ave. New York, NY 10017 (212) 573-8484 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 NIH/National Cancer Institute 9000 Rockville Pike, Bldg. 31, Rm. 1A2A Bethesda, MD 20892 1-800-4-CANCER The National Cancer Institute has developed PDQ (Physician Data Query), a computerized database designed to give the public, cancer patients and families, and health professionals quick and easy access to many types of information vital to patients with this and many other types of cancer. To gain access to this service, call: Cancer Information Service (CIS) 1-800-4-CANCER In Washington, DC and suburbs in Maryland and Virginia, 636-5700 In Alaska, 1-800-638-6070 In Oahu, Hawaii, (808) 524-1234 (Neighbor islands call collect) For genetic information and genetic counseling referrals, please contact: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References MENDELIAN INHERITANCE IN MAN, 8th ed.: Victor A. McKusick; Johns Hopkins University Press, 1986. Pp. 570-71. CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W.B. Saunders Co., 1988. Pp. 994-98. HEMATOLOGY, 4th Ed.: William J. Williams, et al., Editors; McGraw-Hill, Inc., 1990. Pp. 1005-20.