Division of Hematology and Oncology, University of New Mexico Comprehensive Cancer Center, 1 University of New Mexico, 1201 Camino de Salud, NE Albuquerque, NM, 87102, USA.
Division of Internal Medicine, University of New Mexico School of Medicine, MSC08 4720 1 UNM, Albuquerque, NM, 87131-0001, USA.
Curr Oncol Rep. 2023 Aug;25(8):833-840. doi: 10.1007/s11912-023-01419-z. Epub 2023 Apr 25.
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.
本文总结了目前对毛细胞白血病（HCL）的认识，包括其发病表现、诊断、治疗和监测，以及新兴治疗方法的相关观点。
过去十年中，HCL 的生物学研究取得了巨大进展，这为新型治疗策略的发展奠定了基础。现有管理策略的数据不断成熟，为接受化疗或化疗免疫治疗的患者的治疗结果和预后提供了更深入的了解。嘌呤核苷类似物仍然是治疗的基石，利妥昔单抗的加入加深并延长了初治和复发患者的缓解。靶向治疗在 HCL 的治疗中具有更明确的作用，BRAF 抑制剂现在在某些情况下具有一线治疗的潜力，包括复发患者。识别可靶向突变、评估可测量残留疾病和风险分层的下一代测序继续是活跃的研究领域。HCL 的最新进展导致初治和复发患者的治疗更有效。未来的努力将集中在识别需要强化方案的高危疾病患者上。多中心合作是改善这种罕见疾病的总体生存率和生活质量的关键。
