May-Hegglin异常(MHA)2例及文献复习

Two cases of May-Hegglin abnormality and literature review

目的提高对May-Hegglin异常(MHA)临床、血细胞形态学、生理及病理状态下管理的认识。方法回顾性分析2017年8月就诊于中山市博爱医院的2例MHA患者临床、实验室资料及血细胞形态学结果,并复习相关文献。结果 2例患者血常规均提示血小板明显降低(儿子:19×10~9/L,母亲:9×10~9/L),人工计数分别为57×10~9/L、28×10~9/L,但无皮肤、内脏出血等临床表现,血涂片提示中性粒细胞包浆上可见蓝色的纺锤形杜勒氏小体,血小板偏少,散在分布,巨大血小板多见。结论 MHA经常被忽视,经常被误诊为免疫性血小板减少症。详细询问家族史,仔细评估外周血涂片是重要的步骤,MHA患者大部分无出血的临床表现,儿童疫苗接种、孕妇分娩(凝血功能异常除外)均常规进行,各种手术时给予相应的术前、术后处理可预防出血的发生。

Objective To improve the understanding of May-Hegglin abnormality（MHA）in clinical,blood cell morphology,physiological and pathological conditions.Methods The clinical and laboratory data of 2 cases of MHA and the morphological results of blood cells were analyzed retrospectively,and the related literatures were reviewed.Results The blood routine of 2 patients showed obvious decrease of platelets（son：19×10^9/L,mother：9×10^9/L）,and the platelet count was 57×10^9/L and 28×10^9/L,respectively,but there were no clinical manifestations of skin and internal bleeding.The blood smear showed that the blue spindles of D9 hle-like on the neutrophil plasma were visible.Blood platelet was less and scattered.Large platelets were common.Conclusion MHA is often neglected and misdiagnosed as immune thrombocytopenia.It is important to ask the family history and evaluate the peripheral blood smear carefully.Most MHA patients have no hemorrhagic clinical manifestation.Vaccination of children and delivery of pregnant women（except of abnormal coagulation function）are performed routinely.Corresponding treatment before and after surgery can prevent the incidence of bleeding.