IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli", Bologna, Italy.
Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Università degli Studi, Bologna, Italy.
Am J Hematol. 2021 Oct 1;96(10):1204-1210. doi: 10.1002/ajh.26287. Epub 2021 Jul 23.
The treatment of hairy cell leukemia (HCL) has considerably changed over years. Purine analogues, namely cladribine, now represent the treatment of choice. One hundred and eighty-four patients were followed between 1986 and 2018 and treated according to era-specific guidelines. Responses were classified by combining Consensus Resolution criteria and marrow immunohistochemistry. Patients were grouped according to the number of treatment lines they received. Patients treated first line responded in 86% of cases, with complete response (CR) in 44% of cases. Response rates remained high throughout the first four lines (84%, 81%, 79% for the second line onward, with CR in 38%, 37%, 15% of cases respectively). One hundred and twenty-two patients received cladribine as first line treatment, with a response rate of 86% and a CR rate of 54%. Among the 66 CR patients, 45 (68%) have never received further therapy: 11 patients are in continuous CR between 5 and 10 years after treatment, 14 between 10 and 20 years and three patients at more than 20 years. Median time-to-next treatment (TTNT) for frontline cladribine-treated patients was 8.2 years: partial responders had a significantly shorter median TTNT than CR patients (5.3 years vs median not reached at 25.8 years, p < 0.001). Patients with HCL require subsequent lines of therapy in more than 50% of cases. Purine analogues allow significant response rates when applied first line and upon retreatment. Some patients may enjoy long lasting treatment-free intervals after one course of cladribine.
毛细胞白血病（HCL）的治疗方法多年来发生了很大变化。嘌呤类似物，即克拉屈滨，现在是首选的治疗方法。184 名患者于 1986 年至 2018 年期间接受随访，并根据特定时代的指南进行治疗。反应通过结合共识缓解标准和骨髓免疫组织化学进行分类。患者根据接受的治疗线数进行分组。一线治疗的患者中有 86%有反应，完全缓解（CR）率为 44%。在前四线治疗中，反应率一直很高（二线后分别为 84%、81%和 79%，CR 率分别为 38%、37%和 15%）。122 名患者接受克拉屈滨作为一线治疗，反应率为 86%，CR 率为 54%。在 66 例 CR 患者中，45 例（68%）从未接受过进一步治疗：11 例患者在治疗后 5 至 10 年持续 CR，14 例在 10 至 20 年，3 例在 20 年以上。一线克拉屈滨治疗患者的中位无进展生存期（TTNT）为 8.2 年：部分缓解者的中位 TTNT 明显短于 CR 患者（5.3 年 vs 未达到的中位 25.8 年，p<0.001）。超过 50%的 HCL 患者需要后续治疗线。嘌呤类似物在一线应用和再次治疗时可获得显著的反应率。一些患者在接受一个疗程的克拉屈滨治疗后可能会享受长时间的无治疗间隔。
