Division of Hematology/Oncology, Scripps Clinic, La Jolla, CA; and.
Blood. 2014 Jan 9;123(2):177-83. doi: 10.1182/blood-2013-06-508754. Epub 2013 Nov 5.
Hairy cell leukemia (HCL) is a rare, indolent B-cell disorder in which single courses of cladribine induce high rates of complete responses. We report on 88 young HCL patients (≤40 years of age at diagnosis) treated with cladribine from the Scripps Clinic HCL Database, of whom 83 were evaluable for response. Seventy-three patients (88%) achieved an initial complete response and 10 (12%) a partial response, with a median response duration of 57 months. Forty-eight patients (58%) relapsed, with a median time to first relapse for all responders of 54 months. Eight patients developed 11 second primary malignancies with an excess frequency of 1.60 (95% confidence interval, 0.80-2.89). Thirteen (15%) patients died with a mortality ratio compared with age-matched normals of 1.85 (95% confidence interval, 1.07-3.18). Median overall survival for all patients following the first cladribine course was 231 months, and 251 months from diagnosis. Single courses of cladribine induce high rates of complete and durable responses in the majority of young HCL patients and are therefore recommended for HCL patients regardless of age.
毛细胞白血病（HCL）是一种罕见的、惰性的 B 细胞疾病，单次 cladribine 治疗可诱导很高的完全缓解率。我们报告了来自 Scripps 诊所 HCL 数据库的 88 例年轻 HCL 患者（诊断时≤40 岁）接受 cladribine 治疗的情况，其中 83 例可评估反应。73 例患者（88%）获得了初始完全缓解，10 例（12%）获得了部分缓解，中位缓解持续时间为 57 个月。48 例患者（58%）复发，所有应答者的首次复发中位时间为 54 个月。8 例患者发生了 11 例第二原发恶性肿瘤，其发生率高于正常人群的 1.60（95%置信区间，0.80-2.89）。13 例（15%）患者死亡，与年龄匹配的正常人相比，死亡率为 1.85（95%置信区间，1.07-3.18）。所有患者首次 cladribine 治疗后的中位总生存期为 231 个月，从诊断开始为 251 个月。单次 cladribine 治疗可诱导大多数年轻 HCL 患者获得高完全缓解率和持久缓解，因此无论年龄大小，均推荐 cladribine 治疗 HCL。
