骨髓增生异常综合征伴巨核细胞发育异常3例报告  

Myelodysplastic syndrome with megakaryocyte dysplasia: 3 cases report

作　　者：龚辉[1] 康春香[2] 孟凡凯[3] 刘文励[3] 黄丽芳[3] 

机构地区：[1]深圳市罗湖区人民医院,深圳518000 [2]中山大学附属第八医院深圳福田医院 [3]华中科技大学同济医学院附属同济医院

出　　处：《内科急危重症杂志》2017年第4期281-283,共3页Journal of Critical Care In Internal Medicine

基　　金：基金项目:深科技创新2017(No:131)

摘　　要：目的:探讨骨髓增生异常综合征(MDS)伴巨核细胞发育异常[又称难治性血小板减少型MDS(MDSRT)]患者的临床特点及与特发性血小板减少性紫癜(ITP)的鉴别要点。方法:回顾性分析3例MDS-RT的临床特点、诊治经过、骨髓凃片小巨核细胞酶标(CD41a)免疫组化染色及骨髓细胞遗传学的结果。结果:3例血小板计数显著减少,骨髓像巨核细胞增生伴成熟障碍,其中,2例可见单圆核巨核细胞,临床上初始诊断为ITP,但用激素治疗、甚至环胞素治疗,无明显疗效。最后2例发现细胞遗传学异常,1例骨髓凃片CD41a免疫化学染色发现巨核细胞呈明显的病态造血,诊断为MDS-RT。结论:对难治性血小板减少性紫癜患者,应做骨髓活检,骨髓巨核细胞CD41a或CD42b免疫化学染色,细胞遗传学检查,注意与MDS-RT的鉴别。Objective To investigate the clinical characteristics of patients with myelodysplastic syndrome（ MDS）-refractory thrombocytopenia（ RT） and the differences between RT and idiopathic thrombocytopenic purpura（ ITP）. Methods： A retrospective analysis was performed on the clinical characteristics,treatment,the presence of bone marrow smear of small megakaryocytes enzyme staining with specific antibodies（ CD41a） and cytogenetic features of MDS-RT patients. Results Of the 3 cases,all had significant thrombocytopenia and megakaryocyte hyperplasia with maturation disorder in the bone marrow,and mono-round-nuclear megakaryocytes were seen in 2 cases. At first,they were diagnosed as having ITP clinically,but insensitive to glucocorticoid and cyclosporine. Lastly,they were diagnosed as having MDS-RT,including 2 cases identified with cytogenetic abnormalities,and 1 case with dysplasia by bone marrow biopsy and smear of small megakaryocytes enzyme staining with specific antibodies（ CD41a）. Conclusion： For RT patients,it is suggested to undertake bone marrow biopsy,small megakaryocytes enzyme staining with specific antibodies（ CD41 a or CD42b） and cytogenetic examination to distinguish from MDS-RT.

关 键 词：骨髓增生异常综合征 巨核细胞发育异常 细胞遗传学 免疫组织化学染色 

分 类 号：R551.3[医药卫生—血液循环系统疾病]