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2015 ; 22
(4
): 355-61
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Hairy cell leukemia: update and current therapeutic approach
#MMPMID26049757
Salam L
; Abdel-Wahab O
Curr Opin Hematol
2015[Jul]; 22
(4
): 355-61
PMID26049757
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PURPOSE OF REVIEW: In this review, we discuss the pathogenesis and standard
therapeutic approach to hairy cell leukaemia (HCL) as well as newer targeted
therapies under investigation showing promising end-points in treating HCL.
RECENT FINDINGS: HCL is an indolent B-cell leukaemia. Historically, HCL patients
have achieved excellent response to purine nucleoside analogues and single purine
analogue treatment with pentostatin or cladribine is currently the standard of
care for initial treatment. Most patients achieve complete remission with this
form of therapy. However, long-term follow-up has demonstrated that a large
number of patients eventually develop relapsed disease. Relapse disease tends to
be more difficult to treat and refractory to the same purine analogues.
Development of relapsing and refractory disease after initially achieving
complete remission with purine analogue treatment has generated a need for
alternative therapies. SUMMARY: Identification of the BRAFV600E mutation in
nearly 100% of HCL patients has provided rationale for inclusion of BRAF
inhibitors into the therapeutic armamentarium to treat HCL. Clinical trials are
currently underway measuring efficacy of vemurafenib in achieving clinical
response in relapsed/refractory HCL and also toxicity. Other novel therapies with
monoclonal and immunotoxin-conjugated antibodies have also shown promising
response in recent investigational studies.
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