TY - JOUR
T1 - Management of Relapsed Hairy Cell Leukemia
T2 - A Systematic Review of Novel Agents and Targeted Therapies
AU - Siddiqui, Raheel
AU - Sardar, Muhammad
AU - Shahzad, Moazzam
AU - Jose, Jemin
AU - Selene, Insija
AU - Shah, Zunaira
AU - Qureshi, Anum
AU - Shafqat, Madeeha
AU - Kashif, Rimsha
AU - Ahmad, Maheen
AU - Mejia-Garcia, Alex
AU - Anwer, Faiz
N1 - Publisher Copyright:
© 2021 Elsevier Ltd
PY - 2021/10
Y1 - 2021/10
N2 - 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.
AB - 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.
KW - ibrutinib
KW - moxetumomab pasudotox
KW - Refractory
KW - rituximab
KW - vemurafenib
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U2 - 10.1016/j.clml.2021.06.007
DO - 10.1016/j.clml.2021.06.007
M3 - Review article
C2 - 34275772
AN - SCOPUS:85110445989
SN - 2152-2650
VL - 21
SP - 659
EP - 666
JO - Clinical Lymphoma, Myeloma and Leukemia
JF - Clinical Lymphoma, Myeloma and Leukemia
IS - 10
ER -