Hairy cell leukemia: towards a curative strategy.
Although not all patients who have HCL require therapy at diagnosis, most eventually need treatment. Historically, splenectomy and interferon-alpha resulted in hematologic responses; however, responses tend to be short. The introduction of purine analogs dramatically changed the prognosis for most patients who hae 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 prolong DFS. Although both agents yield the same rates of CR and survival, 2-CdA seems easier to administer and my 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 denied 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.
Gidron
Adi
A
Department of Medicine, Division of Hematology/Oncology, Northwestern University Feinberg School of Medicine, 676 North St. Clair, Suite 850, Chicago IL, 60611 USA.
Tallman
Martin S
MS
D000970:Antineoplastic Agents / Q000627:therapeutic use*; D018572:Disease-Free Survival; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D018365:Neoplasm, Residual; D012008:Recurrence; D012074:Remission Induction; D013156:Splenectomy / Q000379:methods
D000970:Antineoplastic Agents