文章：

对279例毛细胞白血病（HCL）患者队列的分析：10年随访

Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up

原文发布日期：2020-05-27

DOI: 10.1038/s41408-020-0328-z

类型: Article

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英文摘要：

In total, 279 patients with hairy-cell leukemia (HCL) were analyzed, with a median follow-up of 10 years. Data were collected up to June 2018. We analyzed responses to treatment, relapses, survival, and the occurrence of second malignancies during follow-up. The median age was 59 years. In total, 208 patients (75%) were treated with purine analogs (PNAs), either cladribine (159) or pentosatin (49), as the first-line therapy. After a median follow-up of 127 months, the median overall survival was 27 years, and the median relapse-free survival (RFS) was 11 years. The cumulative 10-year relapse incidence was 39%. In patients receiving second-line therapy, the median RFS was 7 years. For the second-line therapy, using the same or another PNA was equivalent. We identified 68 second malignancies in 59 patients: 49 solid cancers and 19 hematological malignancies. The 10-year cumulative incidences of cancers, solid tumors, and hematological malignancies were 15%, 11%, and 5.0%, respectively, and the standardized incidence ratios were 2.22, 1.81, and 6.67, respectively. In multivariate analysis, PNA was not a risk factor for second malignancies. HCL patients have a good long-term prognosis. PNAs are the first-line treatment. HCL patients require long-term follow-up because of their relatively increased risk of second malignancies.
 

摘要翻译： 

本研究共分析了279例毛细胞白血病（HCL）患者，中位随访时间为10年，数据收集截止至2018年6月。我们分析了治疗反应、复发情况、生存率及随访期间继发恶性肿瘤的发生情况。患者中位年龄为59岁，共208例（75%）接受嘌呤类似物（PNA）作为一线治疗，其中克拉屈滨159例，喷司他丁49例。中位随访127个月后，患者中位总生存期为27年，中位无复发生存期（RFS）为11年。10年累计复发率为39%。接受二线治疗的患者中位RFS为7年，且使用相同或另一种PNA进行二线治疗效果相当。共59例患者发生68例次继发恶性肿瘤，包括49例实体癌和19例血液系统恶性肿瘤。10年累计实体癌、实体瘤和血液系统恶性肿瘤发生率分别为15%、11%和5.0%，标准化发病比分别为2.22、1.81和6.67。多变量分析显示PNA并非继发恶性肿瘤的危险因素。结论表明：HCL患者长期预后良好，PNA是一线治疗首选，但因继发恶性肿瘤风险相对升高，患者需接受长期随访。

原文链接：

Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up