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102例以红系增生为特征的骨髓增生异常综合征患者的临床分析

[Clinical analysis in a cohort of 102 patients with myelodysplastic syndrome characterized by erythroid hyperplasia].

作者信息

Yu Y, Sun A N, Chen S N, Wang Q R, Zhang T T, Wu D P

机构信息

Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Collaborative Innovation Center of Hematology, the First Affiliated Hospital of Soochow University, Suzhou 215006, China.

出版信息

Zhonghua Nei Ke Za Zhi. 2017 Jan 1;56(1):29-33. doi: 10.3760/cma.j.issn.0578-1426.2017.01.008.

DOI:10.3760/cma.j.issn.0578-1426.2017.01.008
PMID:28056320
Abstract

To investigate the clinical and laboratorial characteristics of patients with myelodysplastic syndrome (MDS) and erythroid hyperplasia. MDS patients whose bone marrow was hypercellular with erythroid lineage more than 50% and blasts account for less than 20% of non-erythroid cells were enrolled in this study. The ratio of mature erythrocytes to nucleated erythrocytes was no more than 20, namely MDS patients with erythroid hyperplasia(MDS-E). The retrospective analysis comprised 102 patients with MDS-E from the First Affiliated Hospital of Suzhou University. Clinical characteristics, karyotype, and the prognostic significance of erythroid hyperplasia were evaluated. A total of 48 MDS-E patients (47.1%) presented a variety of cytogenetic abnormalities. The most frequently involved chromosomes were chromosome 8 (39.5% of all abnormal karyotypes), chromosome 7 (22.9%), followed by chromosome 5 (18.8%), chromosome 1 (16.7%) and chromosome 20 (16.7%). Hemoglobin (Hb) level affected the prognosis by survival analysis. The overall survival (OS) of MDS-E patients with Hb equal or more than 70 g/L was longer than that of patients less than 70 g/L (<0.001). Allogeneic hematopoietic stem cell transplantation (HSCT) significantly improved the OS compared with best supportive care (<0.001) and chemotherapy (<0.001). The extent of erythroid hyperplasia in bone marrow did not impact on prognosis (=0.187). Compared with previous reports of MDS patients, MDS-E patients have higher level of erythroid hyperplasia, more common erythroid dyshematopoiesis, more frequent 8 and 1 chromosome abnormalities. The degree of erythroid hyperplasia is not correlated with prognosis. Allogeneic hematopoietic stem cell transplantation improves the prognosis.

摘要

探讨骨髓增生异常综合征(MDS)合并红系增生患者的临床及实验室特征。本研究纳入骨髓细胞增多且红系比例超过50%、原始细胞占非红系细胞比例小于20%的MDS患者。成熟红细胞与有核红细胞的比例不超过20,即红系增生的MDS患者(MDS-E)。对苏州大学附属第一医院的102例MDS-E患者进行回顾性分析。评估临床特征、核型及红系增生的预后意义。共48例(47.1%)MDS-E患者存在多种细胞遗传学异常。最常受累的染色体为8号染色体(占所有异常核型的39.5%)、7号染色体(22.9%),其次为5号染色体(18.8%)、1号染色体(16.7%)和20号染色体(16.7%)。生存分析显示血红蛋白(Hb)水平影响预后。Hb水平等于或高于70 g/L的MDS-E患者总生存期(OS)长于低于70 g/L的患者(<0.001)。与最佳支持治疗(<0.001)和化疗(<0.001)相比,异基因造血干细胞移植(HSCT)显著改善了OS。骨髓红系增生程度对预后无影响(P=0.187)。与既往MDS患者报道相比,MDS-E患者红系增生水平更高,红系造血异常更常见,8号和1号染色体异常更频繁。红系增生程度与预后无关。异基因造血干细胞移植可改善预后。

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