TY - JOUR
T1 - The pharmacological management of hairy cell leukemia
AU - Ramos Perez, Jorge
AU - Ravandi-Kashani, Farhad
N1 - Publisher Copyright:
© 2020 Informa UK Limited, trading as Taylor & Francis Group.
PY - 2020/7/23
Y1 - 2020/7/23
N2 - Introduction: Hairy cell leukemia (HCL) is a B-cell lymphoid malignancy that accounts for approximately 2% of all leukemias. Treatment with purine nucleoside analogs (PNA) results in a high response rate and remains the standard of care. Long term follow-up shows that most patients relapse and require retreatment. Newer combination strategies and agents have emerged to try to reduce the relapse rate and to address cases of PNA refractoriness. Areas covered: The authors reviewed the literature on the pharmacological management of HCL, including recent studies that led to new agents being incorporated into practice. Expert opinion: Combination of cladribine plus rituximab produces a high rate of measurable residual disease-negative complete remission. In our center, newly diagnosed patients are offered cladribine followed by 8 weekly doses of rituximab in an ongoing phase II trial. Patients in first relapse are also offered this combination if they were initially treated with a single-agent PNA, or if the remission duration was ≥5 years after first-line cladribine plus rituximab. Patients who relapse within 5 years are offered therapy with a novel agent that may include the BRAF inhibitor vemurafenib, alone or in combination with rituximab, dabrafenib in combination with trametinib, the BTK inhibitor ibrutinib, or moxetumomab pasudotox.
AB - Introduction: Hairy cell leukemia (HCL) is a B-cell lymphoid malignancy that accounts for approximately 2% of all leukemias. Treatment with purine nucleoside analogs (PNA) results in a high response rate and remains the standard of care. Long term follow-up shows that most patients relapse and require retreatment. Newer combination strategies and agents have emerged to try to reduce the relapse rate and to address cases of PNA refractoriness. Areas covered: The authors reviewed the literature on the pharmacological management of HCL, including recent studies that led to new agents being incorporated into practice. Expert opinion: Combination of cladribine plus rituximab produces a high rate of measurable residual disease-negative complete remission. In our center, newly diagnosed patients are offered cladribine followed by 8 weekly doses of rituximab in an ongoing phase II trial. Patients in first relapse are also offered this combination if they were initially treated with a single-agent PNA, or if the remission duration was ≥5 years after first-line cladribine plus rituximab. Patients who relapse within 5 years are offered therapy with a novel agent that may include the BRAF inhibitor vemurafenib, alone or in combination with rituximab, dabrafenib in combination with trametinib, the BTK inhibitor ibrutinib, or moxetumomab pasudotox.
KW - Hairy Cell Leukemia
KW - cladribine
KW - pharmacological management
KW - rituximab
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U2 - 10.1080/14656566.2020.1754397
DO - 10.1080/14656566.2020.1754397
M3 - Review article
C2 - 32378970
AN - SCOPUS:85084482727
SN - 1465-6566
VL - 21
SP - 1337
EP - 1344
JO - Expert opinion on pharmacotherapy
JF - Expert opinion on pharmacotherapy
IS - 11
ER -