Perioperative haemostatic management of Glanzmann thrombasthenia for abdominal surgery.

Glanzmann thrombasthenia is a rare congenital platelet disorder characterized by spontaneous mucocutaneous bleeding and severe bleeding complications during major surgery. This report centres on the perioperative haemostatic management of a patient with Glanzmann thrombasthenia undergoing elective major abdominal surgery. The treatment regimen was based mainly on recombinant activated factor VII, fibrinogen, and factor XIII, reducing platelet transfusion to a minimum. No red blood cell transfusions were needed perioperatively. For haemostatic monitoring, routine laboratory tests were sufficient.