单纯巨核细胞再生障碍临床分析

Pure Megakaryocytic Aplasia:A Clinical Study of Seven Cases

目的总结单纯巨核细胞再生障碍的临床特点,提高诊治水平。方法血液常规分析;骨髓涂片经瑞氏染色后细胞分类。结果骨髓涂片上无巨核细胞或巨核细胞显著减少,血小板计数在48×109/L以下,平均为22×109/L;出血症状明显,伴程度不同的贫血,少数有肝脾肿大。经治疗,3例痊愈,2例死亡,2例无变化。结论单纯巨核细胞再生障碍临床与血液学表现与原发性血小板减少性紫癜(ITP)有某些相似,注意二者的鉴别诊断有助于临床治疗与疾病的转归。

Objective To summarize the clinical characteristics of pure mcgakaryocytic aplasia to improve diagnosis and treatment. Methods Blood conventional analysis was performed and cells were classified after bone marrow smears were stained by Wright＇s staining in 7 patients with pure mcgakaryocytic aplasia. Results The bone marrow smears didn＇ t show any mcgakaryocytc or marked decrease of mcgakaryocytc ; The blood platelet counts were below 48 × 10^9/L with an average of 22× 10^9/L; Severe hemorrhagic symptoms appeared with various degrees of anemia in all patients and hcpatomcgaly and splcnomcgaly were observed in a few patients. After treatment ,3 patients recovered,2 patient died, and no improvement was seen in 2. Conclusion The clinical and hematological manifestations of pure mcgakaryocytic aplasia arc similar to primary thrombocytopcnic purpura, and differential diagnosis of the two will contribute to better clinical treatment and turnover of symptoms.