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65例骨髓增生异常综合征患者实验室及临床特征分析

[Analysis on laboratory and clinical characteristics in 65 cases of myelodysplastic syndrome].

作者信息

Chen Bao-An, Gao Chong, Ding Jie, Ding Jia-Hua, Sun Yun-Yu, Zhao Gang, Cheng Jian, Wang Jun, Bao Wen, Song Hui-Hui, Xia Guo-Hua, Ma Jin-Long, Wu Lan-Lan

机构信息

Department of Hematology, Southeast University Medical College, Nanjing 210009, Jiangsu Province, China.

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2009 Dec;17(6):1472-6.

Abstract

The aim of this study was to gain more insight into the understanding of myelodysplastic syndrome in the clinical and laboratory features. The clinical data of 65 patients with MDS were reviewed and analysed. According to FAB criteria, 65 patients were classified as follows: 27 patients with RA, 1 patient with RAS, 33 patients with RAEB, 3 patients with RAEB-T, and 1 patient with CMML. The median age of them was 66 years old (range 19-89 years), and 6 patients had a history of toxic exposure (secondary MDS). The bone marrow smears, bone marrow biopsy and cytogenetic examinations were performed in this study. The results showed that dysplasia was found in 64 patients examined with bone marrow smears (98.5%), among them trilineage dysplasia in 21 patients (32.3%), bilineage dysplasia in 33 patients (50.8%), only erythroid dysplasia in 8 cases (12.3%) and 2 patients (3.1%) only with myeloid dysplasia. The bone marrow biopsy was performed in 38 patients, abnormal localization of immature precursor (ALIP) occurred in 6 cases. 29 patients had abnormal karyotypes, accounting for 59.2% of the 49 patients subjected cytogenetic examination. The abnormal chromosome was the major cytogenetic abnormality, which occurred more often in secondary MDS and the patients with RAEB or RAEB-T. Among the 49 patients who had received cytogenetic examination, 15 patients transformed into AML with the incidence of 30.61%, but only 3 out of 20 patients in the group of normal chromosome transformed into AML (15%), while 12 out of 29 patients in the group of abnormal karyotypes transformed into AML (41.4%). The median time of following up was 35 months (range 2 - 106 months). The median survival time was 26.8 months and 8 months in the patients with normal karyotype and chromosome aberrations respectively. In conclusion, the incidence of MDS in our country is younger than that in Western countries, the rate of abnormal chromosome in high risk MDS is higher than that in low risk MDS. Meanwhile, those who have the change of chromosome are related to the transformation of MDS into AML and have shorter survival time than those MDS patients with normal karyotypes.

摘要

本研究的目的是更深入地了解骨髓增生异常综合征的临床和实验室特征。回顾并分析了65例骨髓增生异常综合征患者的临床资料。根据FAB标准,65例患者分类如下:难治性贫血(RA)27例,环形铁粒幼细胞性难治性贫血(RAS)1例,难治性贫血伴原始细胞增多(RAEB)33例,难治性贫血伴原始细胞增多转变型(RAEB-T)3例,慢性粒-单核细胞白血病(CMML)1例。他们的中位年龄为66岁(范围19 - 89岁),6例有中毒暴露史(继发性骨髓增生异常综合征)。本研究进行了骨髓涂片、骨髓活检和细胞遗传学检查。结果显示,64例接受骨髓涂片检查的患者存在发育异常(98.5%),其中三系发育异常21例(32.3%),两系发育异常33例(50.8%),仅红系发育异常8例(12.3%),仅髓系发育异常2例(3.1%)。38例患者进行了骨髓活检,6例出现幼稚前体细胞异常定位(ALIP)。29例患者有异常核型,占接受细胞遗传学检查的49例患者的59.2%。异常染色体是主要的细胞遗传学异常,在继发性骨髓增生异常综合征以及RAEB或RAEB-T患者中更常见。在49例接受细胞遗传学检查的患者中,15例转化为急性髓系白血病(AML),发生率为30.61%,但正常染色体组的20例患者中只有3例转化为AML(15%),而异常核型组的29例患者中有12例转化为AML(41.4%)。中位随访时间为35个月(范围2 - 106个月)。正常核型和染色体畸变患者的中位生存时间分别为26.8个月和8个月。总之,我国骨髓增生异常综合征的发病年龄比西方国家年轻,高危骨髓增生异常综合征的染色体异常率高于低危骨髓增生异常综合征。同时,染色体发生改变者与骨髓增生异常综合征转化为急性髓系白血病有关,且生存时间比核型正常的骨髓增生异常综合征患者短。

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