title: Current Therapy and New Directions in the Treatment of Hairy Cell Leukemia: A Review.
abstract: Hairy cell leukemia (HCL) is a chronic B-cell leukemia noted for an indolent course that ultimately results in cytopenias and massive splenomegaly. Whereas treatment with the nucleoside purine analogues cladribine and pentostatin results in lengthy remissions in nearly all patients with HCL, most patients will experience relapse while a small percentage of patients' disease fails to respond to therapy in the first place. Retreatment with a purine nucleoside analogue often leads to an effective but limited response. For decades, few other viable therapeutic options were available to these patients who required retreatment. Recently, new insights into the mechanism of disease of HCL have led to research in new potential treatment agents, either alone or with a purine nucleoside analogue. Clinical trials with rituximab, bendamustine, and conjugate immunotoxins will reveal what role these therapies will have in HCL treatment. A better understanding of the BRAF/MEK/ERK pathway and the B-cell signaling pathway has allowed further exploration into the novel drugs vemurafenib, dabrafenib, trametinib, and ibrutinib.
authors.affiliation: Division of Internal Medicine, Scripps Green Hospital, La Jolla, California.
authors.forename: Aditya
authors.initials: A
authors.lastname: Sarvaria
authors.affiliation: Division of Hematology/Oncology, Scripps Clinic, La Jolla, California.
authors.forename: Zheng
authors.initials: Z
authors.lastname: Topp
authors.affiliation: Division of Hematology/Oncology, Scripps Clinic, La Jolla, California.
authors.forename: Alan
authors.initials: A
authors.lastname: Saven
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism; D004059:Diffusion of Innovation; D004351:Drug Resistance; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000188:drug therapy* / Q000235:genetics / Q000266:history / Q000378:metabolism / Q000401:mortality; D058990:Molecular Targeted Therapy / Q000009:adverse effects / Q000266:history / Q000639:trends*; D018579:Patient Selection; D012008:Recurrence; D015398:Signal Transduction / Q000187:drug effects; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor