Abstract
Hairy cell leukemia variant (HCL-V) is a rare B cell lymphoproliferative disorder with a clinical-pathological distinction from the classic form of hairy cell leukemia (HCL-C). HCL-V is more aggressive in nature, has a higher tendency to be refractory to conventional purine analog pharmacotherapies, and leads to a poorer prognosis. Hence, these differing features bring paramount importance to the diagnosis and management of HCL-V. While there is no genetic mutation diagnostic of HCL-V, genetic profiling efforts have identified potential therapeutic targets (i.e., MAP2K1, KDM6A, CREBBP, ARID1A, CCND3, U2AF1, KMT2C) and yielded prognostic markers (i.e., IGHV4-34 rearrangements). To date, combination chemoimmunotherapies, such as cladribine and rituximab, have shown the best results in HCL-V. Future directions include targeted therapies such as moxetumomab pasudotox, ibrutinib, trametinib, and binimetinib and potentially anti-CD22 chimeric antigen receptor T cell therapy. The purpose of this review is to provide an outline of the diagnostic approach and an update on the therapeutic advancements in HCL-V.
Original language | English (US) |
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Pages (from-to) | 99-116 |
Number of pages | 18 |
Journal | Current treatment options in oncology |
Volume | 23 |
Issue number | 1 |
DOIs | |
State | Published - Jan 2022 |
Funding
Julie Tran declares that she has no conflict of interest. Charles Gaulin has owns stock in Agios Pharmaceuticals and Portola Pharmaceuticals, and has received compensation for Life Science Strategy Consulting from DeciBio Consulting. Martin S. Tallman has received research funding from AbbVie, Orsenix, BioSight, GlycoMimetics, Rafael Pharmaceuticals, and Amgen; has received royalties from UpToDate; has received non-financial support from Medscape Live Symposium (EHA Congress), CRTI Workshop, Hammersmith Hospital, Hospital Britanico, Penn State University, Mumbai Hematology Group, German AML Cooperative Group, Emirates Hematology Society, Indy Hematology Review, Vanderbilt University, ASCO Direct Highlights, New Orleans Summer Cancer Meeting, Mayo Clinic, UC Davis, National Comprehensive Cancer Network (NCCN), MCI Summit of the Americas, Rambam Medical Center, and University of Miami; has participated on advisory boards for AbbVie, Daiichi Sankyo, Orsenix, Rigel, Delta-Fly Pharma, Tetraphase Pharmaceuticals, Oncolyze, Jazz Pharmaceuticals, Roche, BioSight, Novartis, Innate Pharma, Kura Oncology, and Syros Pharmaceuticals; and has received compensation for service as a consultant from Medscape Live Symposium (EHA Congress), CRTI Workshop, Prime Oncology, Penn State University, Indy Hematology Review, Targeted Oncology, Vanderbilt University, ASCO Direct Highlights, New Orleans Summer Cancer Meeting, Mayo Clinic, UC Davis, Fire Department of New York (FDNY), NCCN, MCI Summit of the Americas, and University of Miami
Keywords
- Chemotherapy
- Genetic profile
- Hairy cell leukemia
- Hairy cell leukemia variant
- Targeted therapy
ASJC Scopus subject areas
- Pharmacology (medical)
- Oncology