骨髓增生异常综合征伴单系病态造血66例临床特征分析  

Analysis of clinical characteristics of myelodysplastic syndromes with single lineage dysplasia(MDS-SLD) in 66 cases

作　　者：杨永 黄琨[2] YANG Yong;HUANG Kun(Department of Blood Specialty,Soochow Hopes Hematonosis Hospital,Suzhou,Jiangsu 215100,China;Department of Specialty,The First People’s Hospital of Zhangjiagang,Zhangjiagang,Jiangsu 215600,China)

机构地区：[1]苏州弘慈血液病医院血液科,江苏苏州215100 [2]张家港市第一人民医院血液科,江苏张家港215600

出　　处：《安徽医药》2020年第1期106-110,共5页Anhui Medical and Pharmaceutical Journal

摘　　要：目的对照评价骨髓增生异常综合征伴单系病态造血(myelodysplastic syndromes with single lineage dysplasia,MDS⁃SLD)和难治性血细胞减少伴单系病态造血(refractory cytopenia with unilineage dysplasia,RCUD)的临床特征。方法收集2012年至2017年于苏州弘慈血液病医院及张家港市第一人民医院就诊的MDS⁃SLD和RCUD病人病例资料,分析外周血象、骨髓病态造血、染色体异常、预后分组及随访结果。结果共66例MDS⁃SLD,58例符合RCUD分型,另8例无法按照RCUD分型。所有病人外周血均未见原始细胞。MDS⁃SLD和RCUD外周血单系减少分别为27例、25例,两系减少39例、33例。分别有20例、18例合并染色体异常,以预后良好及中等为主。IPSS预后分组中,MDS⁃SLD仅有1例为较高危组,RCUD均属较低危。国际预后积分系统(IPSS⁃R)预后分组中,两组病人均仅有1例为较高危组。6例病人随访期内出现疾病进展,其中1例转为急性髓系白血病(AML)。MDS⁃SLD与RCUD间染色体异常比例、IPSS及IPSS⁃R预后分组各组间差异无统计学意义(均P>0.05)。结论MDS⁃SLD和RCUD病人外周血表现为单系或两系减少均常见,但部分病例血细胞减少与骨髓病态造血不完全一致,按照RCUD标准无法分组,但能够符合修订后的MDS⁃SLD标准。MDS⁃SLD和RCUD临床特征、预后类似,RCUD和无法按照RCUD分类的单系病态造血或单系/两系血细胞减少的MDS可以归为MDS⁃SLD。Objective To evaluate the clinical characteristics of myelodysplastic syndromes with single lineage dysplasia(MDS⁃SLD)and refractory cytopenia with unilineage dysplasia(RCUD).Methods Clinical data of MDS⁃SLD and RCUD patients were collect⁃ed in Soochow Hopes Hematonosis Hospital and the First People’s Hospital of Zhangjiagang from 2012 to 2017,and peripheral blood counts,morphological myelodysplasia,chromosomal abnormalities,prognosis classification and follow⁃up results were retro⁃spectively analyzed.Results There were 66 MDS⁃SLD cases in total,including 58 RCUD cases and 8 unclassified patients.No blasts were found in peripheral blood in all cases.Also in peripheral blood,27 MDS⁃SLD cases and 25 RCUD cases were unilin⁃eage cytopenia,while bilineage cytopenia were detected in 39 MDS⁃SLD cases and 33 RCUD cases respectively.Chromosomal ab⁃normalities were found in 20 MDS⁃SLD cases and 18 RCUD cases.Most of them were good and intermediate karyotypes.Only one MDS⁃SLD patient was in higher⁃risk group,while all the RCUD patients were in lower⁃risk group according to IPSS prognosis classi⁃fication.In IPSS⁃R prognosis classification,only one patient in both groups was found in the higher⁃risk group.Six cases progressed during follow⁃up,one of which developed to AML.There were no statistically significance in the percentage of abnormal chromosome between MDS⁃SLD and RCUD groups and in the differences between IPSS and IPSS⁃R groups(P>0.05).Conclusions Unilin⁃eage cytopenia and bilineage cytopenia in peripheral blood were common in MDS⁃SLD and RCUD patients,but cytopenia in some cases do not entirely consist with myelodysplasia.Some unclassified cases by RCUD criteria could meet the revised diagnostic crite⁃ria of MDS⁃SLD.The clinical characteristics and prognosis are similar in MDS⁃SLD and RCUD.RCUD,unclassified single lineage dysplasia or MDS with unilineage or bilineage cytopenia could be classified as MDS⁃SLD.

关 键 词：骨髓增生异常综合征 全血细胞减少 造血系统 染色体畸变 骨髓增生异常综合征伴单系病态造血 难治性血细胞减少伴单系病态造血 病态造血 

分 类 号：R55[医药卫生—血液循环系统疾病]