Urato A C, Repke J T
Brigham and Women's Hospital, Boston, Massachusetts, USA.
Am J Obstet Gynecol. 1998 Jul;179(1):260-1. doi: 10.1016/s0002-9378(98)70283-2.
May-Hegglin anomaly is a rare, autosomal dominant disorder characterized by thrombocytopenia and a variable bleeding tendency. In almost all the case reports in the recent literature, platelet transfusion and cesarean section were performed to avoid maternal and neonatal bleeding. We present a case of a woman with May-Hegglin anomaly who had no history of a bleeding tendency. She had a vaginal delivery and a platelet count of 16,000/mm3; the neonate's platelet count was 35,000/mm3. There were no complications. We advocate a reappraisal of basing platelet transfusion and mode of delivery on the platelet count in patients with May-Hegglin anomaly.
May-Hegglin异常是一种罕见的常染色体显性疾病，其特征为血小板减少和不同程度的出血倾向。在近期文献中几乎所有的病例报告里，均进行了血小板输注和剖宫产以避免母婴出血。我们报告一例患有May-Hegglin异常的女性病例，该患者既往无出血倾向病史。她经阴道分娩，血小板计数为16,000/mm³；新生儿血小板计数为35,000/mm³。未出现并发症。我们主张重新评估根据May-Hegglin异常患者的血小板计数来决定是否进行血小板输注及分娩方式。
