$Unique_ID{BRK03934} $Pretitle{} $Title{Leukemia, Hairy Cell} $Subject{Leukemia Hairy Cell Leukemic Reticuloendotheliosis Leukemias Letterer-Siwe Disease } $Volume{} $Log{} Copyright (C) 1986, 1988, 1990, 1991, 1992, 1993 National Organization for Rare Disorders, Inc. 269: Leukemia, Hairy Cell ** IMPORTANT ** It is possible the main title of the article (Hairy Cell Leukemia) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Leukemic Reticuloendotheliosis Information on the following diseases can be found in the Related Disorders section of this report: Leukemias Letterer-Siwe Disease 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. 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 (pancytopenia). Symptoms Onset of Hairy Cell Leukemia is usually gradual. Symptoms include vague abdominal pain, a feeling of abdominal fullness, malaise, fatigue, weakness, weight loss and easy bruising. A heavy infiltration of red pulp and sinuses of the spleen by hairy cells occurs. Similar hairy cells infiltrate bone marrow, lymph nodes and liver. The disorder may be chronic. If the spleen is removed (splenectomy) a long survival rate is common. In some cases the onset of Hairy Cell Leukemia may be acute. In these cases the prognosis may not be as good as with the chronic type. Causes The cause of Hairy Cell Leukemia is unknown. Affected Population Hairy Cell Leukemia affects about 6,000 persons in the United States. Most of the patients are males of fifty years old or older. Related Disorders Leukemias are the generalized cancerous (neoplastic) disorders of the blood forming tissues, such as bone marrow. They primarily affect tissues forming the white blood cells (leukocytes). There are many different types of Leukemias. Letterer-Siwe disease (Abt-Letterer-Siwe disease, or Systemic Aleukemic Reticuloendotheliosis) is an autosomal recessive hereditary disorder characterized by generalized enlargement of lymph nodes, liver and spleen, combined with a persistent, spiking, low-grade fever. Symptoms include pallor, discrete yellowish-brown spotty elevated (maculopapular) lesions, sometimes with ulceration. Therapies: Standard Until recently, treatment of Hairy Cell leukemia consisted of surgically removing the spleen. The drug methotrexate (with leucovorin as an antidote against its toxic effects) was commonly used to treat this disorder. Glucocorticoids (i.e., for vasculitic symptoms), and alkylating agents are other commonly used drugs. The orphan drug Alpha Interferon has been approved by the FDA for use in treatment of Hairy Cell Leukemia. The drug is manufactured by Hoffman- LaRoche and Schering Plough. Interferon is a hormone naturally produced by the body to fight viral infections. Clinical trials conducted prior to FDA approval indicate that two years after treatment with Alpha Interferon was begun, 92%-94% of treated patients were alive, compared to fewer than 50% of patients treated with conventional therapies. The disease went into remission in 75% to 90% of patients treated with Alpha Interferon. Treatment with Alpha Interferon may include daily injections for up to 6 months, followed by maintenance injections 3 times weekly. Side effects mimic flu-like symptoms which diminish over time. The orphan drug Nipent has been approved by the FDA for patients who do not respond to ordinary therapy for Hairy Cell Leukemia. The drug is manufactured by Warner-Lambert. The drug Leustatin (cladribine or 2CDA) has been approved for treatment of HCL. This drug has proved to be an effective drug in the therapy of Hairy Cell Leukemia. Therapies: Investigational Interleukin-2 has been used on an experimental basis to restore natural killer cell activity in Hairy Cell Leukemia. The drug deoxycoformycin is being used in clinical trials on patients with Hairy Cell Leukemia. Information about obtaining this drug can be located through the Comprehensive Cancer Center (Dr. Eric Kraut), Ohio State University, Columbus, Ohio, or the Investigational Drug Branch of the National Cancer Institute in Bethesda, Maryland. This disease entry is based upon medical information available through March 1993. 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 Hairy-Cell Leukemia, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Hairy Cell Leukemia Foundation P.O. Box 72 Newtonville, MA 02160 (617)-244-8478 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 Recombinant Alpha-2 Interferon in the Treatment of Hairy Cell Leukemia: Thompson JA et al.: Cancer Treatment Rep 69 (7-8): 791-3 (1985 Jul-Aug). Splenectomy for Hairy Cell Leukemia; A Clinical Review of 63 Patients: Van Norman AS et al.: Cancer 57 (3): 644-8 (1986 Feb 1). Therapeutic Options in Hairy Cell Leukemia: Groopman JE: Seminaries in Oncology 12 (4 Suppl 5): 30-4 (1985 Dec). LASTING REMISSIONS IN HAIRY-CELL LEUKEMIA INDUCED BY A SINGLE INFUSION OF 2-CHLORODEOXYADENOSINE, Piro, Lawrence, et al.; N Eng J Med, (April 19, 1990, issue 322 (16)). Pp. 1117-1121.