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Hairy cell leukaemia: review of treatment
Author(s) -
Kurzrock Razelle,
Talpaz Moshe,
Gutterman Jordan U.
Publication year - 1991
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1991.tb08112.x
Subject(s) - hairy cell , deoxycoformycin , medicine , hairy cell leukemia , bone marrow , splenectomy , peripheral blood mononuclear cell , immunology , alpha interferon , gastroenterology , interferon , leukemia , spleen , adenosine deaminase , adenosine , biochemistry , in vitro , chemistry
The cardinal features of hairy cell leukaemia are: (i) cytopenias, (ii) splenomegaly, and (iii) mononuclear cells of B‐cell origin with cytoplasmic projections and tartrate‐resistant acid phosphatase‐positivity. The most common complication is infection. In the past, the mainstay of therapy has been splenectomy, and this procedure is still often suggested as a first‐line approach. However, research during the last decade has resulted in three new, highly effective therapies for hairy cell leukaemia: interferon‐alpha (IFN‐alpha), 2′‐deoxycoformycin (DCF) and 2‐chlorodeoxyadenosine (2CDA). IFN‐alpha is currently approved for this indication. About 90% of patients have a durable haematologic recovery, and complete remission rates range from < 5% to > 40% in different series. It should be noted that patients with partial remissions generally have normal or near‐normal blood counts, and can live indefinitely without disease‐related problems, despite a few remaining hairy cells in the bone marrow. In this paper we will discuss the various therapeutic modalities available for patients with hairy cell leukaemia.