Coombs阳性的骨髓增生异常综合征1例诊断思考

摘要

目的:探讨以自身免疫性溶血为早期表现的骨髓增生异常综合征(MDS)的临床特点以及对MDS的早期诊断思考.方法:回顾性分析1例以自身免疫性溶血为早期表现的MDS患者的临床特点、诊治经过及疾病发展过程.结果:该患者以自身免疫性溶血为早期表现,用足量糖皮质激素治疗1个月效果不佳,复查骨髓细胞学,通过骨髓病态分析提示红系显著病态造血,小巨核酶标染色体提示巨核系统发育异常,患者病情发展迅速,1个月后即转化为急性髓系白血病.结论:MDS可能以溶血为早期表现,进行骨髓细胞病态造血的分析对MDS的诊断十分重要.CD41的小巨核酶标染色有助于早期发现巨核细胞病态改变,帮助MDS的早期诊断.%Objective:To investigate the clinical characteristics of myelodysplastic syndromes (MDS) with autoimmune hemolysis as early manifestation and to discuss the early diagnosis of MDS.Methods:The clinical features,diagnosis and treatment of 1 case of MDS with autoimmune hemolysis as early manifestation were analyzed retrospectively.Results:The patient was characterized with autoimmune hemolyis early,and treated with enough glucocorticoid for one month,but there was no response.Reexamination of bone marrow cytology suggested morphologic dyshematopoiesis in erythroid.The patient transformed rapidly into acute myeloid leukemia after one month.Conclusion:MDS may present with pure hemolysis during early stage.Analysis of bone marrow cells of dysplastic hematopoiesis in the diagnosis of MDS is very important.CD41 immune staining can improve the efficiency of micromegakaryocyte detection,and help the early diagnosis of MDS.