Platelet function, ultrastructure, and survival in the May-Hegglin anomaly

The May-Hegglin anomaly is one of the rare forms of hereditary thrombocytopenia. Since fewer than 100 cases of May-Hegglin anomaly have been described to date, major controversies regarding the adequacy of hemostasis in vivo and abnormalities of platelet function in vitro continue to prevail. This report describes the results of coagulation, platelet function, platelet ultrastructure, and survival studies performed for a family with 6 previously unreported cases occurring in 3 generations. One member of this family also had frequent epistaxis and a prolonged bleeding time associated with cyanotic heart disease that required open heart surgery for correction. The laboratory assessment of hemostasis in such patients before major surgery is also discussed.