title: Advances in therapy for hairy cell leukemia. A review.
abstract: BACKGROUND
  Hairy cell leukemia (HCL) is a chronic B-cell malignancy, typically seen in middle-aged men, characterized by pancytopenia, splenomegaly, immunologic abnormalities, and morphologically typical neoplastic mononuclear cells in the blood, bone marrow, liver, spleen, and other tissues. Diagnosis is confirmed by demonstration of hairy cells in biopsy specimens from the bone marrow or spleen or in peripheral blood. The natural history of this lymphoproliferative disorder varies. Patients may die early during the initial phase of therapy; others may require no therapy; and for some, splenectomy alone, without further treatment, may suffice for many years. Recently, the nucleosides pentostatin (2'-deoxycoformycin) (DCF) and 2'-chlorodeoxyadenosine (2-CdA) have been shown to produce greater numbers of durable complete remissions with curative potential in patients with HCL. The treatment options, with emphasis on major therapeutic advances with alpha-interferon, DCF, and 2-CdA, are reviewed in this article.
  
  
  METHODS
  Studies on HCL published from 1958 to 1992 were reviewed using the Cancerline and Medline retrieval systems and other bibliographies.
  
  
  RESULTS
  Management of HCL has changed in the last decade as a result of three new effective agents: alpha-interferon DCF, and 2-CdA. DCF has produced an overall response rate of 86% and a complete remission rate of 62%. 2-CdA has yielded an overall response rate of 95% and a complete remission rate of 82%. Alpha-interferon has given an overall response rate of 82% and a complete remission rate of 8%. Other agents with limited activities include chlorambucil, cyclophosphamide, cytarabine, vincristine, doxorubicin, and zorubicin hydrochloride. The effects of lithium carbonate, immunotherapy, splenic irradiation, androgens, and leukaphoresis are minimal and transient.
  
  
  CONCLUSIONS
  Modern management of HCL with 2-CdA and DCF is now potentially curative rather than palliative in some patients; however, the optimal therapeutic approach remains uncertain. Alpha-interferon has been approved by the Food and Drug Administration as the first-line drug therapy, followed by DCF in non-responding patients. 2-CdA remains an experimental therapy, but its higher response rate and ease of administration may make it the first-line treatment of choice. Additional research into the biology of HCL and further clinical trials are needed to determine the optimal treatment strategy for this disorder. Therefore, the best therapeutic approach at the current time is to include patients with HCL in ongoing clinical trials.
authors.affiliation: Department of Hematology, University of Texas M.D. Anderson Cancer Center, Houston 77030.
authors.forename: I A
authors.initials: IA
authors.lastname: Jaiyesimi
authors.forename: H M
authors.initials: HM
authors.lastname: Kantarjian
authors.forename: E H
authors.initials: EH
authors.lastname: Estey
mesh_terms: D015762:2-Chloroadenosine / Q000031:analogs & derivatives / Q000627:therapeutic use; D003839:Deoxyadenosines / Q000627:therapeutic use; D005544:Forecasting; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D007372:Interferons / Q000627:therapeutic use; D007943:Leukemia, Hairy Cell / Q000473:pathology / Q000628:therapy*; D008297:Male; D015649:Pentostatin / Q000627:therapeutic use; D013156:Splenectomy; D014740:Vidarabine / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D003839:Deoxyadenosines; D016179:Granulocyte Colony-Stimulating Factor; D015762:2-Chloroadenosine; D015649:Pentostatin; D007372:Interferons; D014740:Vidarabine; C055994:2-chloro-3'-deoxyadenosine; C024352:fludarabine