Service des Maladies du Sang, CHU d'Angers, Angers, France.
Laboratoire d'Hematologie Biologique, CHU de Caen, Caen, France.
Blood Cancer J. 2020 May 27;10(5):62. doi: 10.1038/s41408-020-0328-z.
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.
总计分析了 279 例毛细胞白血病（HCL）患者，中位随访时间为 10 年。数据收集截至 2018 年 6 月。我们分析了治疗反应、复发、生存和随访期间第二恶性肿瘤的发生情况。中位年龄为 59 岁。共有 208 例（75%）患者接受嘌呤类似物（PNAs）治疗，一线治疗药物为克拉屈滨（159 例）或喷司他丁（49 例）。中位随访 127 个月后，中位总生存期为 27 年，中位无复发生存期（RFS）为 11 年。累积 10 年复发率为 39%。接受二线治疗的患者中位 RFS 为 7 年。对于二线治疗，使用相同或另一种 PNA 等效。我们在 59 例患者中发现了 68 例第二恶性肿瘤：49 例实体瘤和 19 例血液系统恶性肿瘤。癌症、实体瘤和血液系统恶性肿瘤的 10 年累积发生率分别为 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 不是第二恶性肿瘤的危险因素。HCL 患者具有良好的长期预后。PNAs 是一线治疗。由于 HCL 患者发生第二恶性肿瘤的风险相对增加，需要长期随访。
