单纯巨核细胞再生障碍临床研究.docVIP

单纯巨核细胞再生障碍临床研究【摘要】 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。 【关键词】巨核细胞；再生障碍；血小板 Pure Megakaryocytic Aplasia:A Clinical Study of Seven Cases LIU Sheng-ping.Nanan Branch,the Second People’s Hospital of Yibin,Sichuan 644000,China 【Abstract】 Objective To summarize the clinical characteristics of pure megakaryocytic 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 megakaryocytic aplasia.Results The bone marrow smears didn?t show any megakaryocyte or marked decrease of megakaryocyte ; The blood platelet counts were below 48×109/L with an average of 22×109/L; Severe hemorrhagic symptoms appeared with various degrees of anemia in all patients and hepatomegaly and splenomegaly 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 megakaryocytic aplasia are similar to primary thrombocytopenic purpura,and differential diagnosis of the two will contribute to better clinical treatment and turnover of symptoms. 【Key words】Megakaryocyte;Aplasia;Blood platelet 单纯巨核细胞再生障碍(pure megakaryocytic aplasia)系无巨核细胞性血小板减少性紫癜(amegakaryocytic thrombocytopenic purpura),低巨核细胞性血小板减少症(hypomegakaryocytic thrombocytopenia)的同义语，属于一种特殊类型的再生障碍性疾病，以巨核细胞数量显著减少或缺乏，临床有不同程度的出血、贫血为主要特征，国内曾有多篇报道，但其诊断标准和治疗转归很不一致。现将四川省宜宾市第二人民医院南岸分院自1995年至2007年收治的该病7例资料作回顾性分析，并对其诊断标准及临床意义进行讨论分析。 1 资料与方法 1．1 一般资料 患者为四川省宜宾市第二人民医院南岸分院从1995年至2007收治的住院患者6例，门诊患者1例，男5例，女2例；年龄2~63岁，平均32．5岁。5例因不同原因出血就诊，2例因咳嗽、发热就诊。 1．2 症状与体征 7例患者中，全部病例均有出血史。皮肤瘀斑紫癜者6例，牙龈出血4例，鼻衄者2例，眼结膜下出血1例，血尿2例，便血1例；7例中5例有轻、中、重程度不同的贫血，3例肝肿大，2例脾肿大。 1．3 实验室检查 7例患者分别检查血常规分析，仪器：F820半自动全血细胞分析仪、CA50全自动