Document Detail

Hairy cell leukaemia.
MedLine Citation:
PMID:  7913637     Owner:  NLM     Status:  MEDLINE    
Hairy cell leukaemia is a rare chronic lymphoproliferative disease, characterized by splenomegaly, pancytopenia and recurrent infection. The characteristic 'hairy cells', present in the peripheral blood and bone marrow, are the hallmark of this leukaemia. The disease has a chronic, progressive course, and the majority of patients afflicted by it require therapy. The most common reason to initiate treatment is neutropenia with or without associated infectious complications, or the development of severe thrombocytopenia. Therapeutic options in hairy cell leukaemia include splenectomy, interferon administration, or the use of chemotherapeutic agents such as pentostatin (2'-deoxycoformycin) and 2-chlorodeoxyadenosine. Splenectomy is still indicated in the treatment of young patients with significant splenomegaly and only minimal bone marrow involvement. Interferon treatment induces remission in approximately 90% of patients with hairy cell leukaemia, but complete remission is obtained in only 5-10%. The development of antibodies against interferon was initially considered a major problem, but longer follow-up of patients who developed antibodies has shown that it is transient and does not have a significant impact on the overall response to treatment. Pentostatin induces complete remission in 60-70% of patients and partial remission in 20-40%. 2-Chlorodeoxyadenosine is a very promising drug in the treatment of this rare leukaemia, inducing long-lasting complete remission in approximately 80% of patients. While interferon does not cure the disease, it is possible that a subset of patients treated with pentostatin or 2-chlorodeoxyadenosine are cured. Longer follow-up of these patients will determine whether this is true.
L C Platanias; H M Golomb
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Baillière's clinical haematology     Volume:  6     ISSN:  0950-3536     ISO Abbreviation:  Baillieres Clin. Haematol.     Publication Date:  1993 Dec 
Date Detail:
Created Date:  1994-08-25     Completed Date:  1994-08-25     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8800474     Medline TA:  Baillieres Clin Haematol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  887-98     Citation Subset:  IM    
Division of Hematology-Oncology, Loyola University Chicago-Stritch School of Medicine, Maywood, IL 60153.
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MeSH Terms
Cladribine / therapeutic use
Clinical Trials as Topic
Immunologic Deficiency Syndromes / etiology
Immunologic Factors / therapeutic use
Interferon-alpha / therapeutic use
Leukemia, Hairy Cell* / complications,  epidemiology,  pathology,  therapy
Middle Aged
Pancytopenia / etiology
Pentostatin / therapeutic use
Remission Induction
Splenomegaly / etiology,  surgery
Treatment Outcome
Reg. No./Substance:
0/Immunologic Factors; 0/Interferon-alpha; 4291-63-8/Cladribine; 53910-25-1/Pentostatin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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