$Unique_ID{BRK03933} $Pretitle{} $Title{Leukemia, Chronic Myelogenous} $Subject{Leukemia, Chronic Myelogenous Chronic Myeloid Leukemia Chronic Granulocytic Leukemia Chronic Myelocytic Leukemia GML CGL Polycythemia Vera Myelofibrosis-Osteosclerosis} $Volume{} $Log{} Copyright (C) 1989, 1990, 1991, 1992, 1993 National Organization for Rare Disorders, Inc. 695: Leukemia, Chronic Myelogenous ** IMPORTANT ** It is possible that the main title of the article (Chronic Myelogenous Leukemia) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Chronic Myeloid Leukemia Chronic Granulocytic Leukemia Chronic Myelocytic Leukemia GML CGL Information on the following diseases can be found in the Related Disorders section of this report: Polycythemia Vera Myelofibrosis-Osteosclerosis 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 myelogenous leukemia is characterized by an excessive amount of white blood cells in the bone marrow, spleen, liver and blood. As the disease progresses, the leukemic cells invade other areas of the body including the intestinal tract, kidneys, lungs, gonads and lymph nodes. There are two phases to chronic myelogenous leukemia. The first phase, or chronic phase, is characterized by an overproduction of white blood cells. An advanced phase is called the acute phase or blast crisis. At this point, over 50% of the cells in the bone marrow are immature malignant cells (blast cells or promelocytes). In the acute phase, the leukemia is very aggressive and does not respond well to therapy. Approximately 85% of all patients with chronic myelogenous leukemia enter the acute phase. Symptoms Many patients with chronic myelogenous leukemia show nonspecific symptoms at the time of diagnosis. The most common symptoms are fatigue, weakness, itchiness, night sweats, abdominal discomfort or weight loss. An enlarged spleen is usually discovered upon physical examination. Chronic myelogenous leukemia is commonly diagnosed when a patient is undergoing blood tests for some other reason. When the acute phase of chronic myelogenous leukemia occurs, the patient experiences severe weight loss, high fever, bone pain, enlargement of the liver and spleen, pain in the joints (arthralgia), excess calcium in the blood and hemorrhages appearing as patches of purplish discoloration on the skin and mucous membranes. Causes The exact cause of chronic myelogenous leukemia is not known. It is believed that in some cases excessive exposure to radiation increases an individual's chances of developing the disease. Blood samples of patients with chronic myelogenous leukemia show the presence of abnormal and useless cells that reproduce more rapidly than normal cells. Ninety percent of these neoplastic cells show a consistent rearrangement of chromosomes. This rearrangement is the result of a transference of genetic material from chromosome 22 to chromosome 9 and vice versa. As a result of this transference, chromosome 22 ends up shorter than normal. This shortened chromosome is known as the Philadelphia chromosome, and the possibility that it plays a role in the onset of the disease or in the reproduction of neoplastic cells is being investigated. Affected Population Chronic myelogenous leukemia is slightly more prevalent in males than in females. It may occur at any age, but predominately appears between the 30's and 40's. Related Disorders Symptoms of the following disorders can be similar to those of chronic myelogenous leukemia. Comparisons may be useful for a differential diagnosis: Polycythemia Vera is a chronic disorder of the bone marrow. It is characterized by an increase in the number of red blood cells (erythrocytosis) and hemoglobin concentration in the blood. The initial symptoms are fatigue, drowsiness, itchiness and dizziness. (For more information on this disorder, choose "Polycythemia" as your search term in the Rare Disease Database). Myelofibrosis-Osteosclerosis is a disorder characterized by the growth of fibrous tissue in the bone marrow causing anemia, weakness and fatigue due to constant replacement of normal bone marrow cells. Episodes of severe pain in the abdomen, the bones and joints also may occur. (For more information on this disorder, choose "Myelofibrosis" as your search term in the Rare Disease Database). There are many other types of leukemia that may mimic chronic myelogenous leukemia. The leukemias are malignant disorders of white blood cells. Therapies: Standard Treatment of chronic myelogenous leukemia includes radiation therapy to the spleen and chemotherapy. The objective is to reduce both the spleen size and the white blood cell count, inducing a remission. The most commonly prescribed drugs are hydroxyurea or busulfan. Since decreasing the bone marrow's production of white blood cells does not ordinarily change the natural course of chronic myelogenous leukemia toward the acute stage, more aggressive therapies are also being tried. These include other chemotherapeutic drugs, early removal of the spleen, and bone marrow transplantation. The most success has been achieved with bone marrow transplantation. The best recipients for bone marrow transplantation are under the age of 40. Transplantation must be done before the onset of the acute phase of chronic myelogenous leukemia, since successful results of marrow transplantation after the acute phase has begun are not as high. The acute phase of chronic myelogenous leukemia proceeds aggressively and rapidly. Some patients seem to respond temporarily to vincristine and prednisone therapy, but in general chemotherapy may be ineffective. There is less chance of a second remission during the acute phase. The FDA recently approved the orphan drug Idamycin (Idarubicin hydrochloride) for use in the treatment of Chronic Myelogenous Leukemia. Idarubicin is manufactured by Adria Laboratories. Other treatment is symptomatic and supportive. Therapies: Investigational Interferon Alfa-2a, Recombinant (Roferon-A) is a new orphan drug being used in the treatment of Chronic Myelogenous leukemia (CML). It is manufactured by Hoffman-LaRoche, Inc., 340 Kingsland St., Nutley, NJ 07110. Of ninety-six patients treated with this therapy, remission occurred in seventy for up to a period of thirty months. Amgen, Inc., 1840 Dehavilland Dr., Thousand Oaks, CA, 91320, is developing an orphan drug to be used in the treatment of myelodysplastic syndromes. The name of the drug is Recombinant-Methionyl Granulocyte-Colony Stimulating Factor (Neupogen). The Office of Orphan Products Development gave a New Grant Award to Dr. Walter A. Blatter in 1990 for his work with immunotherapy of Myelcid Leukemia using Anti-9-blocked Ricin Immunoconjugate. Dr. Blatter is with ImmunoGen, Inc. of Cambridge, MA. For acute attacks of CML, Medarex, Inc., 12 Commerce Ave., West Lebanon, NH, 03784, has developed the biologic Monoclonal Antibody PM81. Idarubicin (Idamycin) is a new orphan product being used in the treatment of Chronic Myelogenous Leukemia. The drug is manufactured by: Adria Laboratories P.O. Box 16529 Columbus, OH 42316-6529 This disease entry is based upon medical information available through April 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 Myelogenous Leukemia, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Leukemia Society of America 733 Third Avenue 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) References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 1081-1083. THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D. ed.-in-chief; Merck, Sharp & Dohme Laboratories., 1982. Pp. 755-756. BLAST CRISIS Of PHILADELPHIA CHROMOSOME-POSITIVE CHRONIC MYELOCYTIC LEUKEMIA (CML). B. Anger et al; BLUT, (Sep. 1988; 57(3):131-7. BONE MARROW TRANSPLANTATION FOR CHRONIC MYELOGENOUS LEUKEMIA IN CHRONIC PHASE. J.M. Goldman et al; ANN INTERN MED, (Jun 1988; 108(6):806-14. INTERFERON-ALPHA PRODUCES SUSTAINED CYTOGENIC RESPONSES IN CHRONIC MYELOGENOUS LEKUEMIA., M. Talpaz, et al., Ann Intern Med, (April 1, 1991, issue 114). Pp. 532-538.