Everlien M, Knoch K, Farah I, Greve H
Klink für Herz- und Thoraxchirurgie, Klinikum Krefeld.
Dtsch Med Wochenschr. 2001 Jan 19;126(3):47-9. doi: 10.1055/s-2001-10359.
Twelve years prior to hospitalization because of a severe bleeding, a 70 year old patient was diagnosed with a May-Hegglin anomaly, which is a rare autosomal dominant inherited form of thrombocytopenia.
The blood smear contained signs of the May-Hegglin anomaly: Döhle's inclusion bodies and giant platelets. Platelet counts were around 30 G/l. Coronary angiography revealed a highly severe left main stenosis.
Even using maximum drug therapy, angina pectoris could not be stabilized. Therefore emergency coronary artery bypass grafting had to be performed. Before skin incision 3 micrograms/kg DDAVP (Desmopressin) were administered and after extracorporeal circulation 8 units of platelets were transfused. In addition, perioperative coagulation management was performed according to usual standards. There were no bleeding complications. The patient could leave the clinic after 11 days in stable condition.
Patients showing May-Hegglin anomaly, even with serious thrombocytopenia, can be operated using extracorporal circulation without a high risk of bleeding.
在因严重出血住院前12年，一名70岁患者被诊断为May-Hegglin异常，这是一种罕见的常染色体显性遗传性血小板减少症。
血涂片显示有May-Hegglin异常的迹象：杜勒小体和巨大血小板。血小板计数约为30×10⁹/L。冠状动脉造影显示左主干高度严重狭窄。
即使使用最大剂量药物治疗，心绞痛仍无法稳定。因此必须进行急诊冠状动脉搭桥术。皮肤切开前给予3微克/千克去氨加压素（DDAVP），体外循环后输注8单位血小板。此外，围手术期凝血管理按常规标准进行。无出血并发症。患者在11天后病情稳定出院。
患有May-Hegglin异常的患者，即使血小板严重减少，也可在体外循环下进行手术，且出血风险不高。
