Hairy Cell Leukemia: Towards a Curative Strategy

Adi Gidron, Martin S. Tallman*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

7 Scopus citations

Abstract

Although not all patients who have HCL require therapy at diagnosis, most eventually need treatment. Historically, splenectomy and interferon-under(α, ̇) resulted in hematologic responses; however, responses tend to be short. The introduction of purine analogs dramatically changed the prognosis for most patients who have HCL. It is now considered standard of care to use a purine analog, such as 2-CdA or 2′-DCF, as first-line therapy. This approach results in a high CR rate and prolonged DFS. Although both agents yield the same rates of CR and survival, 2-CdA seems easier to administer and may be associated with less toxicity. Despite the excellent results with purine analogs, most patients have MRD detected by sensitive techniques; 30% to 40% of patients eventually relapse and most require further therapy. A repeat course of 2-CdA (or 2′-DCF) will result in CR in approximately 70% of patients. For patients who have relapsed or refractory disease, monoclonal antibody-based therapies are emerging options. Rituximab and BL22 are highly active in this setting. Until BL22 becomes widely available, rituximab is a reasonable choice for salvage therapy; however, the dosing schedule needs to be defined further. The roles of rituximab and BL22 as initial therapy for patients who have previously untreated HCL have not been investigated. Fig. 1 is a suggested algorithm for the treatment of HCL. With the introduction of effective new agents, further studies will determine whether the now achievable prolonged survival will translate into cure.

Original languageEnglish (US)
Pages (from-to)1153-1162
Number of pages10
JournalHematology/Oncology Clinics of North America
Volume20
Issue number5
DOIs
StatePublished - Oct 2006
Externally publishedYes

ASJC Scopus subject areas

  • Hematology
  • Oncology

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