TY - JOUR
T1 - Hairy Cell Leukemia
T2 - Towards a Curative Strategy
AU - Gidron, Adi
AU - Tallman, Martin S.
PY - 2006/10
Y1 - 2006/10
N2 - 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.
AB - 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.
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U2 - 10.1016/j.hoc.2006.06.004
DO - 10.1016/j.hoc.2006.06.004
M3 - Review article
C2 - 16990114
AN - SCOPUS:33748589200
SN - 0889-8588
VL - 20
SP - 1153
EP - 1162
JO - Hematology/Oncology Clinics of North America
JF - Hematology/Oncology Clinics of North America
IS - 5
ER -