本文概述了毛细胞白血病 (HCL) 关于表现、诊断、治疗和监测的现状，并提出了新的治疗方案。在过去的十年中，对 HCL 生物学的理解取得了巨大进展，促进了新的治疗策略的发展。现有治疗策略的数据成熟度也为使用化疗或化疗免疫疗法的患者的治疗效果和预后提供了深度的洞见。嘌呤核苷类似物仍然是治疗的核心，利妥昔单抗的使用深化并延长了一线治疗和复发治疗的反应。针对性疗法在 HCL 管理中有了更明确的角色，BRAF 抑制剂在特定情况下有可能成为一线治疗和复发治疗的选择。寻找可靶向突变、评估可测残留疾病和风险分层的下一代测序技术仍是积极研究的领域。最近在 HCL 领域取得的进展，使一线治疗和复发治疗方案更有效。未来的努力将集中于识别需要强化治疗方案的高危患者。多中心合作对改善这种罕见病的总生存率和生活质量至关重要。© 2023. The Author(s).

This article summarizes the current state of knowledge of hairy cell leukemia (HCL) regarding presentation, diagnosis, therapy, and monitoring, including perspectives on emergent therapies.Over the past decade, there has been enormous progress in the understanding of the biology of HCL which has led to the development of novel therapeutic strategies. The maturation of data regarding existing management strategies has also lent considerable insight into therapeutic outcomes and prognosis of patients treated with chemo- or chemoimmunotherapy. Purine nucleoside analogs remain the cornerstone of treatment, and the addition of rituximab has deepened and prolonged responses in the upfront and relapsed setting. Targeted therapies now have a more defined role in the management of HCL, with BRAF inhibitors now having a potential in the first-line setting in selected cases as well as in relapse. Next-generation sequencing for the identification of targetable mutations, evaluation of measurable residual disease, and risk stratification continue to be areas of active investigation. Recent advances in HCL have led to more effective therapeutics in the upfront and relapsed setting. Future efforts will focus on identifying patients with high-risk disease who require intensified regimens. Multicenter collaborations are the key to improving overall survival and quality of life in this rare disease.© 2023. The Author(s).