Abstract
Background: Hairy cell leukemia (HCL) responds well to purine analogs with an overall median relapse free survival of 11-16 years. Most patients can be retreated with the same or a different purine analog however a subset of patients will become resistant or develop cumulative toxicities. Novel agents such as Vemurafenib (BRAF kinase inhibitor), Bendamustine/Rituximab (BR), Moxetumomab pasudotox (anti CD-22 recombinant immunotoxin) and Ibrutinib have emerging roles in patients with relapsed HCL. Methods: Five databases (PubMed, Embase, Cochrane Library, Web of Science and ClinicalTrials.gov) were searched using the following search terms: “hairy cell leukemia” or “leukemia, hairy cell” AND “relapse” or “recurrence”. We included only prospective clinical trials with outcome data. Results: Vemurafenib monotherapy was evaluated in two separate arms of a phase 2 trial. In the US arm (n=24), the ORR was 100% (CR 42%; PR 58%). In the Italian arm (n=26), the ORR was 96% (CR 35%; PR 62%). In a phase 2 study (n=25), the combination of vemurafenib and rituximab showed CR of 100%. The combination of BR achieved an ORR of 100% whereas CR was 50% and 67% at a bendamustine dose of 70mg/m2 (n=6) and 90 mg/m2 (n=6) respectively. In a phase 3 trial, moxetumomab pasudotox (n=80) had an ORR of 75% (CR 41%). Single agent Ibrutinib (n=37) had an ORR of 54%. Therapies were generally well tolerated. Conclusion: Novel agents have good efficacy in HCL in patients with multiple relapses.
Original language | English (US) |
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Pages (from-to) | 659-666 |
Number of pages | 8 |
Journal | Clinical Lymphoma, Myeloma and Leukemia |
Volume | 21 |
Issue number | 10 |
DOIs | |
State | Published - Oct 2021 |
Externally published | Yes |
Keywords
- ibrutinib
- moxetumomab pasudotox
- Refractory
- rituximab
- vemurafenib
ASJC Scopus subject areas
- Hematology
- Oncology
- Cancer Research