Table of Contents
1
UI - 12057121
AU - Savoie L; Johnston JB
TI -
Hairy cell leukemia.
SO - Curr Treat Options Oncol 2001 Jun;2(3):217-24
AD - CancerCare Manitoba, 675 McDermot Avenue, Winnipeg, Manitoba, Canada,
R3E0V9.
The standard therapy for hairy cell leukemia (HCL) is with the
nucleoside analogs, 2"-deoxycoformycin (dCF) or 2-chlorodeoxyadenosine
(CdA), which produce morphologic complete remissions (CRs) in the
majority of patients, although residual hairy cells can frequently be
detected by molecular or immunologic techniques. Relapses continue to
occur over time, but most patients respond well to retreatment with the
same agent. The longest follow-up is for patients treated with dCF,
where the 5- and 10-year relapse-free survival rates are 80% to 85% and
67% to 76%, respectively. dCF is usually administered as 4 mg/m2
intravenously every second week until CR followed by two additional
treatments for consolidation. CdA is administered as 0.09 mg/kg/d x 7,
by continuous intravenous infusion, although it may be equally effective
when given as daily boluses or subcutaneously. More recent studies have
suggested that CdA, 0.15 mg/kg intravenously weekly x 6, produces
equivalent response rates, while reducing the risk of febrile
neutropenia (which occurs in approximately 50% of patients using the
standard regimen). We have found this to be a very simple, safe, and
effective regimen. Both dCF and CdA should be used with caution in the
presence of renal or hepatic dysfunction, and both are contraindicated
in the presence of active infection. Interferon-alfa (3 x 10(6) U
subcutaneously three times per week for 12 months) produces inferior
response rates but is less likely to cause febrile neutropenia. It can
be considered for initial treatment for patients with active infection,
patients at high risk of febrile neutropenia, and patients who cannot
tolerate or are resistant to the nucleoside analogs. Splenectomy is now
rarely performed in HCL, but it is required for splenic rupture and may
be of value in "splenic" HCL or those with massive splenomegaly and
hypersplenism. In preliminary studies, monoclonal antibodies directed
against CD20 or CD25 also show activity in HCL, but their roles in this
disease require further study.
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