Perioperative management of patients with hemophilia

Hemophilia A and hemophilia B are X chromosome-linked congenital bleeding disorders caused by a deficiency or absence of activity of coagulation factor VIII (hemophilia A) or factor IX (hemophilia B), which are graded in different degrees of severity (mild, moderate, severe). Depending on the severity patients may experience spontaneous bleeding episodes or will develop excessive bleeding in the context of injuries or surgery. Hemophilia should not be a contraindication for an invasive procedure; however, a number of conditions are required to provide successful surgery and an uncomplicated and safe postoperative course. This review provides an overview of hemophilia and the key biochemical laboratory and clinical aspects as well as possible specific and non-specific treatment options and addresses the special needs for the perioperative care of these patients.