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 could not be stabilized. Therefore emergency coronary artery bypass grafting had 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 performed according to usual standards. There were no bleeding complications. The patient could leave the clinic after 11 days in stable condition. Conclusion: Patients showing May-Hegglin anomaly, even with serious thrombocytopenia, can be operated using extracorporal circulation without a high risk of bleeding.