title: Hairy cell leukaemia: review of treatment.
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.
authors.affiliation: Department of Clinical Immunology and Biological Therapy, University of Texas M.D. Anderson Cancer Center, Houston 77030.
authors.forename: R
authors.initials: R
authors.lastname: Kurzrock
authors.forename: M
authors.initials: M
authors.lastname: Talpaz
authors.forename: J U
authors.initials: JU
authors.lastname: Gutterman
mesh_terms: D015762:2-Chloroadenosine / Q000031:analogs & derivatives / Q000627:therapeutic use; D017338:Cladribine; D003839:Deoxyadenosines / Q000627:therapeutic use; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D007943:Leukemia, Hairy Cell / Q000628:therapy*; D015649:Pentostatin / Q000627:therapeutic use; D013156:Splenectomy
chemical_list: D003839:Deoxyadenosines; D016898:Interferon-alpha; D016179:Granulocyte Colony-Stimulating Factor; D015762:2-Chloroadenosine; D015649:Pentostatin; D017338:Cladribine