Coronary artery bypass grafting in May-Hegglin anomaly

History: 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 Fare autosomal dominant inherited form of thrombocytopenia. Investigations: The blood smear contained signs of the May-Hegglin anomaly: Dohle's inclusion bodies and giant platelets. Platelet counts were around 30 G/I. Coronary angiography revealed a highly severe left main stenosis. Treatment and course: Even using maximum drug therapy, angina pectoris coul d not be stabilized. Therefore emergency coronary artery bypass grafting ha d to be performed. Before skin incision 3 mug/kg DDAVP (Desmopressin) were administered and after extracorporeal circulation 8 units of platelets were transfused. In addition, perioperative coagulation management was performe d according to usual standards. There were no bleeding complications. The p atient could leave the clinic after 11 days in stable condition. Conclusion: Patients showing May-Hegglin anomaly, even with serious thrombo cytopenia, can be operated using extracorporal circulation without a high r isk of bleeding.