Faculty Collaboration Database

Hairy cell leukaemia: review of treatment. Br J Haematol 1991 Oct;79 Suppl 1:17-20

Date

10/01/1991

Pubmed ID

1718389

DOI

10.1111/j.1365-2141.1991.tb08112.x

Scopus ID

2-s2.0-0025937961 (requires institutional sign-in at Scopus site)   25 Citations

Abstract

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 less than 5% to greater than 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.

Author List

Kurzrock R, Talpaz M, Gutterman JU

Author

Razelle Kurzrock MD Center Associate Director, Professor in the Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

2-Chloroadenosine
Cladribine
Deoxyadenosines
Granulocyte Colony-Stimulating Factor
Humans
Interferon-alpha
Leukemia, Hairy Cell
Pentostatin
Splenectomy