Advances in the Treatment of Hairy Cell Leukemia Variant

Julie Tran*, Charles Gaulin, Martin S. Tallman

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

3 Scopus citations


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 languageEnglish (US)
Pages (from-to)99-116
Number of pages18
JournalCurrent treatment options in oncology
Issue number1
StatePublished - Jan 2022


  • Chemotherapy
  • Genetic profile
  • Hairy cell leukemia
  • Hairy cell leukemia variant
  • Targeted therapy

ASJC Scopus subject areas

  • Pharmacology (medical)
  • Oncology


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