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比较修订版第 4 版(2016 年)和第 5 版(2022 年)世界卫生组织骨髓增生异常肿瘤分类。

Comparison of the revised 4th (2016) and 5th (2022) editions of the World Health Organization classification of myelodysplastic neoplasms.

机构信息

State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.

MDS and MPN Centre, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.

出版信息

Leukemia. 2022 Dec;36(12):2875-2882. doi: 10.1038/s41375-022-01718-7. Epub 2022 Oct 12.

Abstract

We used data from 852 consecutive subjects with myelodysplastic neoplasms (MDS) diagnosed according to the 2016 (revised 4th) World Health Organization (WHO) criteria to evaluate the 2022 (5th) edition WHO classification of MDS. 30 subjects previously classified as MDS with an NPM1 mutation were re-classified as acute myeloid leukaemia (AML). 9 subjects previously classified as MDS-U were re-classified to clonal cytopenia of undetermined significance (CCUS). The remaining 813 subjects were diagnosed as: MDS-5q (N = 11 [1%]), MDS-SF3B1 (N = 70 [9%]), MDS-biTP53 (N = 53 [7%]), MDS-LB (N = 293 [36%]), MDS-h (N = 80 [10%]), MDS-IB1 (N = 161 [20%]), MDS-IB2 (N = 103 [13%]) and MDS-f (N = 42 [5%]) and MDS-biTP53 (N = 53 [7%]). 34 of these subjects came from the 53 (64%) MDS-biTP53 previously diagnosed as MDS-EB. Median survival of subjects classified as MDS using the WHO 2022 criteria was 45 months (95% Confidence Interval [CI], 34, 56 months). Subjects re-classified as MDS-biTP53 and MDS-f had significantly briefer median survivals compared with other MDS sub-types (10 months, [8, 12 months] and 15 months [8, 23 months]). In conclusion, our analyses support the refinements made in the WHO 2022 proposal.

摘要

我们使用了 852 例连续的骨髓增生异常肿瘤(MDS)患者的数据,这些患者是根据 2016 年(修订版 4 版)世界卫生组织(WHO)标准诊断的,以评估 2022 年(5 版)WHO MDS 分类。30 例先前被归类为伴 NPM1 突变的 MDS 的患者被重新归类为急性髓系白血病(AML)。9 例先前被归类为 MDS-U 的患者被重新归类为克隆性血细胞减少症未确定意义(CCUS)。其余 813 例患者被诊断为:MDS-5q(N=11[1%]),MDS-SF3B1(N=70[9%]),MDS-biTP53(N=53[7%]),MDS-LB(N=293[36%]),MDS-h(N=80[10%]),MDS-IB1(N=161[20%]),MDS-IB2(N=103[13%])和 MDS-f(N=42[5%])和 MDS-biTP53(N=53[7%])。其中 34 例来自之前诊断为 MDS-EB 的 53 例 MDS-biTP53 患者。根据 WHO 2022 标准分类的患者的中位生存期为 45 个月(95%置信区间[CI],34,56 个月)。与其他 MDS 亚型相比,重新归类为 MDS-biTP53 和 MDS-f 的患者的中位生存期明显缩短(10 个月,[8,12 个月]和 15 个月,[8,23 个月])。总之,我们的分析支持了 WHO 2022 提案中的改进。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b74/9712101/148b6e1fedc1/41375_2022_1718_Fig1_HTML.jpg

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