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Management of Relapsed Hairy Cell Leukemia: A Systematic Review of Novel Agents and Targeted Therapies.

AbstractBACKGROUND:
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.
AuthorsRaheel Siddiqui, Muhammad Sardar, Moazzam Shahzad, Jemin Jose, Insija Selene, Zunaira Shah, Anum Qureshi, Madeeha Shafqat, Rimsha Kashif, Maheen Ahmad, Alex Mejia-Garcia, Faiz Anwer
JournalClinical lymphoma, myeloma & leukemia (Clin Lymphoma Myeloma Leuk) Vol. 21 Issue 10 Pg. 659-666 (10 2021) ISSN: 2152-2669 [Electronic] United States
PMID34275772 (Publication Type: Journal Article, Systematic Review)
CopyrightCopyright © 2021 Elsevier Ltd. All rights reserved.
Chemical References
  • Antineoplastic Agents
Topics
  • Adolescent
  • Antineoplastic Agents (pharmacology, therapeutic use)
  • Child
  • Female
  • Humans
  • Leukemia, Hairy Cell (drug therapy)
  • Male

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