Hairy Cell Leukemia: Treatment

Hairy Cell Leukemia: overview

This algorithm outlines treatment of classic hairy cell leukemia in two steps, first-line and relapsed or refractory disease. First-line therapy is a purine analog, either cladribine or pentostatin, given with or without rituximab.

At relapse, the chart offers three routes. A repeat purine analog combined with rituximab is shown as a way to achieve a durable second remission. For tumors carrying BRAF V600E, the targeted option is vemurafenib with or without rituximab. For multiply relapsed disease, the antibody-drug conjugate moxetumomab pasudotox is listed. The chart does not link specific trials or trial summaries to these branches, so the overview is limited to the treatment sequence it displays: purine analog first, then retreatment, BRAF-directed therapy or an ADC depending on mutation status and the number of prior relapses.

Trials behind this algorithm

Cladribine in HCL Pagano L et al, Blood Cancer J, 2022; PMID: 35853850

Hairy Cell Leukemia: the algorithm in words

Starting point: Hairy cell leukemia (classic).

First-line (first-line): Purine analog: Cladribine or pentostatin (± rituximab).
Relapsed / refractory (second-line): Repeat: Purine analog + rituximab (durable second remission); BRAF: Vemurafenib ± rituximab (BRAF V600E); ADC: Moxetumomab pasudotox (multiply relapsed).