McDunn S, Hartz W, Ts'Ao C, Green D
Department of Medicine, Northwestern University Medical School, Chicago, Illinois.
Am J Clin Pathol. 1991 May;95(5):715-8. doi: 10.1093/ajcp/95.5.715.
May-Hegglin anomaly (MHA) is a rare hereditary condition that is characterized by cytoplasmic inclusions in leukocytes and giant platelets. Many patients have some degree of thrombocytopenia. Most individuals with MHA are asymptomatic, but 25-43% of patients previously reported have had a hemorrhagic tendency. The authors describe a patient with MHA who had no history of hemorrhage but who developed complete coronary thrombosis after attempted angioplasty despite an apparent platelet count of 24,000 per mm3. Laboratory investigations revealed a normal bleeding time, normal platelet aggregation, and an increase in the size of approximately two-thirds of the platelets. The calculated platelet mass was near normal, which probably explains the thrombosis despite a decrease in platelet numbers. The authors conclude that in some patients with MHA platelets are functionally active both in vivo and in vitro.
May-Hegglin异常（MHA）是一种罕见的遗传性疾病，其特征为白细胞和巨大血小板中存在细胞质包涵体。许多患者有一定程度的血小板减少症。大多数MHA患者无症状，但先前报道的患者中有25% - 43%有出血倾向。作者描述了一名患有MHA的患者，该患者无出血史，但在尝试进行血管成形术后发生了完全性冠状动脉血栓形成，尽管其血小板计数明显为每立方毫米24,000个。实验室检查显示出血时间正常、血小板聚集正常，且约三分之二的血小板大小增加。计算得出的血小板质量接近正常，这可能解释了尽管血小板数量减少但仍发生血栓形成的原因。作者得出结论，在一些MHA患者中，血小板在体内和体外均具有功能活性。
