Hairy cell leukemia (HCL)是起源于成熟B淋巴细胞的一种罕见的缓慢型淋巴细胞白血病亚型。经典HCL和HCL变异体（HCLv）的标准一线治疗包括嘌呤核苷类似物（PNA）治疗，可以选择结合利妥昔单抗治疗。然而，近一半的患者会复发并需要随后的治疗。本文总结了近期在难治和复发的HCL治疗方面取得的进展。我们检索了PubMed和MEDLINE数据库中的英文文章。对2010年到2023年1月的发表进行了仔细研究，搜索词包括毛细胞白血病、BRAF抑制剂、Bruton酪氨酸激酶（BTK）抑制剂、CD20单克隆抗体、复发、难治和变异型。对HCL生物学的不断深入理解使设计出了几种新型的靶向药物，这些药物无需化疗，并在早期临床试验中表现出了鼓舞人心的疗效。新型药物很快将可用于协助PNA治疗效果不佳的HCL和HCLv的标准治疗。特别是，BRAF抑制剂vemurafenib和dabrafenib以及结合利妥昔单抗，彻底改变了对复发或难治疾病患者的治疗。

Hairy cell leukemia (HCL) is a rare subtype of indolent lymphoid leukemia originating from a mature B lymphocyte. The standard first-line treatment for classic HCL, and HCL variant (HCLv), consists of purine nucleoside analogs (PNA), with or without rituximab. However, almost half of patients relapse and require subsequent therapy.This article summarizes recent achievements in the treatment of relapsed and refractory HCL. A literature search was conducted of the PubMed and MEDLINE database for articles in English. Publications from 2010 through January 2023 were scrutinized. The search terms used were hairy cell leukemia in conjunction with BRAF inhibitors, Bruton's tyrosine kinase (BTK) inhibitors, CD20 monoclonal antibodies, relapsed, refractory and variant.The growing understanding of HCL biology has allowed the design of several new, chemotherapy-free targeted drugs which have demonstrated encouraging efficacy in early clinical trials.Novel drugs will soon be available to assist standard therapy for HCL and HCLv among patients with suboptimal results following PNA treatment. In particular, the BRAF inhibitors vemurafenib and dabrafenib, with or without rituximab, have revolutionized treatment of patients with relapsed or refractory disease.