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在世界卫生组织 2016 年提案的背景下对慢性髓单核细胞白血病进行预后评估:一项涉及 280 例患者的多中心研究。

Prognostic assessment for chronic myelomonocytic leukemia in the context of the World Health Organization 2016 proposal: a multicenter study of 280 patients.

机构信息

Servicio de Hematología, Hospital General de Agudos "C Durand", Buenos Aires, Argentina.

Servicio de Hematología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

出版信息

Ann Hematol. 2021 Jun;100(6):1439-1449. doi: 10.1007/s00277-021-04539-3. Epub 2021 May 1.

DOI:10.1007/s00277-021-04539-3
PMID:33932168
Abstract

Knowledge on chronic myelomonocytic leukemia (CMML) patients from Argentina and Brazil is limited. Our series of 280 patients depicted an older age at diagnosis (median 72 years old), 26% of aberrant karyotypes, and a prevalence of myelodysplastic (60%) and CMML-0 subtypes (56%). The median overall survival (OS) was 48.2 months for patients in CMML-0 (Ref.), 24.7 months for those in CMML-1 (HR = 2.0, p = 0.001), and 8.8 months for patients in CMML-2 (HR = 4.6, p < 0.001). In the CMML-0 category, median OS were different between myelodysplastic and myeloproliferative subtypes (63.7 vs 21.2 months, p < 0.001); however, no differences were observed within CMML-1 and CMML-2 subtypes (24.7 vs 23.7 months, p = 0.540, and 9.1 vs 8.2 months, p = 0.160). The prognostic impact of 24 variables and 7 prognostic systems was adjusted to the WHO 2016 after validating their usefulness. Multivariate analysis were performed, and the final model revealed Hb ≥ 8 -< 10g/dL (HR 1.7), Hb < 8g/dL (HR 2.8), poor karyotypes (HR 2.1), WHO 2016-CMML-1 (HR 2.1), and CMML-2 (HR 3.5) as independent adverse clinical parameters in our cohort with a borderline influence of platelets count < 50 × 10/L (HR 1.4). We could validate several scoring systems, the WHO 2016 proposal and its prognostic capability, along with accessible covariates, on predicting the outcome in our series of CMML patients from Latin America.

摘要

来自阿根廷和巴西的慢性髓单核细胞白血病(CMML)患者的相关知识有限。我们的 280 例患者系列研究表明,患者的诊断年龄较大(中位数为 72 岁),存在 26%的异常核型,骨髓增生异常(60%)和 CMML-0 亚型(56%)更为常见。CMML-0 (参照)患者的中位总生存期(OS)为 48.2 个月,CMML-1 患者为 24.7 个月(HR=2.0,p=0.001),CMML-2 患者为 8.8 个月(HR=4.6,p<0.001)。在 CMML-0 类别中,骨髓增生异常和骨髓增生性亚型之间的中位 OS 存在差异(63.7 与 21.2 个月,p<0.001);然而,在 CMML-1 和 CMML-2 亚型中未观察到差异(24.7 与 23.7 个月,p=0.540,9.1 与 8.2 个月,p=0.160)。在验证了其有效性后,对 24 个变量和 7 个预后系统的预后影响进行了调整,以适应 WHO 2016 标准。进行了多变量分析,最终模型揭示了血红蛋白≥8-<10g/dL(HR 1.7)、血红蛋白<8g/dL(HR 2.8)、不良核型(HR 2.1)、WHO 2016-CMML-1(HR 2.1)和 CMML-2(HR 3.5)是我们的队列中独立的不良临床参数,血小板计数<50×10/L(HR 1.4)的影响具有边界性。我们可以验证几个评分系统,包括 WHO 2016 建议及其预后能力,以及可获得的协变量,以预测我们的拉丁美洲 CMML 患者系列的结果。

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