Division of Hematology and Oncology, Scripps Clinic and Scripps MD Anderson, 10666 N. Torrey Pines Ave, La Jolla, CA, 92037, USA.
Scripps Research Translational Institute, La Jolla, CA, USA.
Ann Hematol. 2022 May;101(5):1089-1096. doi: 10.1007/s00277-022-04800-3. Epub 2022 Feb 26.
Hairy cell leukemia (HCL) is a rare hematologic disorder characterized by pancytopenia and splenomegaly for which a single course of cladribine is highly effective in inducing complete remissions. However, there is limited real-world data on outcomes and complications among geriatric patients with HCL treated with cladribine. We conducted a retrospective review of all patients 70 years or older within the Scripps Clinic HCL Database at the time of first treatment with cladribine. Of the 45 patients meeting inclusion criteria, 32 (71%) achieved CR and 4 (9%) achieved PR. Of the 9 remaining patients, 7 achieved normalization of peripheral blood counts after a single course of cladribine (complete hematologic response, CHR) and 2 had no response. The median duration of response for all responders was 119 months. Nine (20%) patients relapsed with a median time to first relapse of 28 months. Ten patients subsequently developed 12 primary malignancies with an excess frequency (observed-to-expected ratio) of 0.85 (95% confidence interval, 0.48-1.49). Median overall survival for the entire cohort was 166 months from time of HCL diagnosis and 119 months from time of first cladribine administration. Forty patient deaths were observed; the standardized mortality ratio (observed-to-expected ratio) was 1.42 (95% confidence interval, 1.03-1.96), representing a statistically significant increase in the risk of death (P = .03). This study supports the high rate of complete and durable responses following a single course of cladribine in geriatric patients.
毛细胞白血病（HCL）是一种罕见的血液系统疾病，其特征为全血细胞减少和脾肿大，用单疗程克拉屈滨治疗可高度有效地诱导完全缓解。然而，在接受克拉屈滨治疗的老年 HCL 患者中，关于结局和并发症的真实世界数据有限。我们对 Scripps 诊所 HCL 数据库中首次接受克拉屈滨治疗时年龄在 70 岁及以上的所有患者进行了回顾性研究。在符合纳入标准的 45 例患者中，32 例（71%）达到完全缓解（CR），4 例（9%）达到部分缓解（PR）。其余 9 例患者中，7 例在接受单疗程克拉屈滨治疗后外周血计数恢复正常（完全血液学缓解，CHR），2 例无反应。所有应答者的中位缓解持续时间为 119 个月。9 例（20%）患者复发，首次复发的中位时间为 28 个月。随后，10 例患者发生了 12 种原发性恶性肿瘤，其发生频率高于预期（观察到的与预期的比值）为 0.85（95%置信区间，0.48-1.49）。整个队列从 HCL 诊断到中位总生存期为 166 个月，从首次克拉屈滨治疗到中位总生存期为 119 个月。观察到 40 例患者死亡；标准化死亡率（观察到的与预期的比值）为 1.42（95%置信区间，1.03-1.96），这表明死亡风险显著增加（P = .03）。这项研究支持在老年患者中，单次克拉屈滨治疗后获得完全和持久缓解的高比率。
