Ibrutininib and Venclexta for PLL – pro
B-cell (B-PLL) and T-cell (T-PLL) prolymphocytic leukemias are rare, poor-prognosis lymphoid neoplasms with similar presentation characterized by symptomatic splenomegaly and lymphocytosis. First-line therapy for B-PLL is with combination purine analog-based chemo-immunotherapy. New B-cell receptor inhibitors, such as ibrutinib and idelalisib, may have a role in the management of B-PLL, especially for the patients harboring abnormalities of TP53.
In regard to Vencelxta, Boidol et al recetly reported a highly T-cell prolymphocytic leukemia (T-PLL)-specific response to the B-cell lymphoma 2 (BCL-2) inhibitor venetoclax in an ex vivo high-throughput drug screen, as well as partial remissions (PRs) in 2 patients with relapsed/refractory (R/R) T-PLL and high disease burden, both of whom were previously treated with multi-agent chemotherapy and alemtuzumab. I did not find similar reports for B-PLL or reprots of ibrutiib and Veclexta used in combination for PLL>.
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Collignon A, et al, Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment. Curr Oncol Rep. 2017 Apr;19(4):29.
Boidol B, Kornauth C, van der Kouwe E, et al. First-in-human response of BCL-2 inhibitor venetoclax in T-cell prolymphocytic leukemia. Blood. 2017;130(23):2499-2503.