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Review
. 2015 Aug 19:8:2147-56.
doi: 10.2147/OTT.S70316. eCollection 2015.

Current and emerging treatment options for hairy cell leukemia

Montserrat López-Rubio  1 , Jose Antonio Garcia-Marco  2
Affiliations
Review

Current and emerging treatment options for hairy cell leukemia

Montserrat López-Rubio et al. Onco Targets Ther. .

Abstract

Hairy cell leukemia (HCL) is a lymphoproliferative B-cell disorder characterized by pancytopenia, splenomegaly, and characteristic cytoplasmic hairy projections. Precise diagnosis is essential in order to differentiate classic forms from HCL variants, such as the HCL-variant and VH4-34 molecular variant, which are more resistant to available treatments. The current standard of care is treatment with purine analogs (PAs), such as cladribine or pentostatin, which provide a high rate of long-lasting clinical remissions. Nevertheless, ~30%-40% of the patients relapse, and moreover, some of these are difficult-to-treat refractory cases. The use of the monoclonal antibody rituximab in combination with PA appears to produce even higher responses, and it is often employed to minimize or eliminate residual disease. Currently, research in the field of HCL is focused on identifying novel therapeutic targets and potential agents that are safe and can universally cure the disease. The discovery of the BRAF mutation and progress in understanding the biology of the disease has enabled the scientific community to explore new therapeutic targets. Ongoing clinical trials are assessing various treatment strategies such as the combination of PA and anti-CD20 monoclonal antibodies, recombinant immunotoxins targeting CD22, BRAF inhibitors, and B-cell receptor signal inhibitors.

Keywords: hairy cell leukemia; ibrutinib; immunotoxins; purine analogs; rituximab; vemurafenib.

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Figures

Figure 1
Figure 1
Algorithm for first-line treatment in hairy cell leukemia. Abbreviations: IV, intravenous; MRD, minimal residual disease; SC, subcutaneous; BM, bone marrow.
Figure 2
Figure 2
Algorithm for second-line treatment in hairy cell leukemia. Notes: Patients who relapse within the first 2 years after initial treatment will receive alternative purine analog plus eight doses of rituximab, while patients who relapse 2 years after first-line treatment will receive the same purine analog plus eight rituximab doses.
See this image and copyright information in PMC

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