Koronare Bypassoperation bei May-Hegglin-Anomalie

Abstract

Patients showing May-Hegglin anomaly, even with serious thrombocytopenia, can be operated using extracorporal circulation without a high risk of bleeding.

Thrombotic events in MYH9 gene-related autosomal macrothrombocytopenias (old May–Hegglin, Sebastian, Fechtner and Epstein syndromes)

“…and 1 ischemic stroke) and two cases of venous thrombosis (a portal vein thrombosis and a deep vein thrombosis). All these patients showed a May-Hegglin pattern [8][9][10][11][12][13][14]. No thrombotic event was found in reported patients with the other MYH9 gene alteration variants (Table 1).…”

Myocardial Infarctions and Other Acute Coronary Syndromes in Rare Congenital Bleeding Disorders

“…The breakdown figures were fibrinogen deficiency: 4 patients; FV deficiency: 2 patients; FVII defects: 2 patients; FXI defect: 36 patients; FXIII: 1 patient 4,5,9 –29 ; and platelet disorders: 8 patients, 1 with GT, 3 with BSS, and 4 with MYH9 defects. 30 –36 The age varied between 27 and 82 (mean age was 60.2). No patient with either FII or FX deficiency was found to meet the inclusion criteria; 7 were female, 39 were male whereas in 6 instances, gender was not reported.…”

Management of May–Hegglin anomaly referred for coronary artery bypass

“…We found only two previous case reports that described a patient with May-Hegglin anomaly who underwent coronary artery bypass graft. In the first case, the patient received desmopressin (3 mg/kg) then 8 units of platelets and aprotinin during extra-corporeal circulation and no bleeding complication was reported [2]. In the second one [3], the patient received aprotinin, 3 units of platelets, 6 packed red blood cells and 1200 cc of fresh frozen plasma.…”