Approach to the patient after relapse of hairy cell leukemia.
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ABSTRACT:
SUBMITTER: Kreitman RJ
PROVIDER: S-EPMC7433026 | biostudies-literature | 2009 Oct
REPOSITORIES: biostudies-literature
Publications
Approach to the patient after relapse of hairy cell leukemia.
Kreitman Robert J RJ Fitzgerald David J P DJ Pastan Ira I
Leukemia & lymphoma 20091001
The current hairy cell leukemia (HCL) treatment is excellent, but evidence of cure with purine analogs cladribine and pentostatin, is lacking. Significant long-term immune suppression, particularly to CD4+ T-cells, and declining complete remission rates with each course, make the identification of new therapies an important goal. The anti-CD20 monoclonal antibody (Mab) rituximab displays significant activity, and, while causing prolonged normal B-cell depletion, spares T-cells. Recombinant immun ...[more]
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Project description:Purpose of reviewIn 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 findingsHCL 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.SummaryIdentification 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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