Hairy cell leukemia is a chronic indolent B-cell lymphoma that can present symptomatically with cytopenias and splenomegaly

Purine analogs- cladribine and pentostatin, are the first-line treatment for hairy cell leukemia. A single course achieves very high complete response rates but leaves patients vulnerable to infectious complications.

However, relapsed and refractory cases do not respond well to purine analogs.The identification of the BRAF V600E mutation has established a novel therapeutic target in hairy cell leukemia . 

Vemurafenib, an oral BRAF inhibitor used to treat late-stage melanoma and Erdheim-Chester disease, has been evaluated in several prospective studies. We therefore conducted a meta-analysis to summarize the available evidence.

 

Overall Response Rate for vemurafenib is high (>91%) and consistent whether  used alone or in combination

Complete Response Rates showed substantial heterogeneity across studies

Combination regimens were associated with substantially higher complete response compared to monotherapy