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1994-01-17
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$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.