意义未明的特发性血细胞减少症临床及实验室检测分析

Clinical and laboratory determination analysis of idiopathic cytopenia with uncertain(undetermined)significance

目的对确诊为意义未明的特发性血细胞减少症(ICUS)患者进行探讨,了解其疾病的发展及归属。方法收集ICUS患者骨髓涂片及外周血涂片、免疫抗原CD34检测、骨髓活检、染色体核型分析、融合基因WT1检测结果并进行综合分析。结果 15例ICUS患者病程均>8个月,表现为1系以上的血细胞减少,有11例是在河北医科大学附属平安医院确诊的,其中2例曾在外院诊断为血小板减少性紫癜,后期因白细胞(WBC)、血红蛋白(Hb)减少,确诊为ICUS,4例是外院确诊后入住本院治疗。15例患者中有1例患者骨髓象表现为红系幼红细胞偶见病态造血,个别患者实验室检查原始细胞数、CD34抗原、融合基因WT1高于参考范围。结论多种疾病可引起血细胞减少,结合临床表现及实验室检查,意义未明的特发性血细胞减少症最终是否进展为典型的骨髓增生异常综合征(MDS)、再生障碍性贫血(AA)或其他血细胞减少性疾病,目前仍需进行临床动态观察。

Objective To investigate idiopathic cytopenia with uncertain（undetermined） significance （ICUS） and its development. Methods ICUS patients were observed for their bone marrow smears and peripheral blood smears. CD34 antigen,bone marrow biopsy,karyotype analysis and fusion gene WT1 were also determined. Results The durations of 15 ICUS patients were〉8 months,and they showed a series of blood cell reducing. There were 11 cases diagnosed in Hebei Medical University Ping＇an Hospital. The 2 cases of them had been diagnosed as idiopathic thrombocytopenic purpura in other hospitals,and then they were diagnosed as ICUS because of white blood cell（WBC） and hemoglobin（Hb） reducing later. The other 4 cases were treated in Hebei Medical University Ping＇an Hospital after being diagnosed in other hospitals. Some of the 15 patients＇ bone marrow showed occasional dyshaematopoiesis in erythroid normoblast. Some patients＇ primitive cells,CD34 antigen and fusion gene WT1 were higher than related reference ranges. Conclusions Various diseases can lead to blood cell reducing. Combined with clinical manifestations and laboratory examinations,whether ICUS will have progress to typical myelodysplastic syndrome（MDS）,aplastic anemia（AA）,or other blood cell reducing diseases,it still requires dynamic clinical observation.