Haemophilia A in a major trauma patient

Abstract

Haemophilia A is a coagulopathy with an absence or functional deficiency of coagulation factor VIII. The disease can be subdivided into a congenital form (incidence 1:10,000-1:20,000) and an acquired form (incidence 1:1,000,000). Both forms present an increased risk of bleeding even in low-impact trauma in severe cases of haemophilia A. The course of a man with haemorrhagic shock after a fall from a height of more than 3 m is described. After the secondary survey in the intensive care unit, several interventions (laparotomy, mass transfusion) were performed to stop the bleeding. A history of haemophilia was belatedly reported by relatives. The activity of factor VIII was determined, and substitution was initiated. No recurrent signs of bleeding occurred. The patient survived in good condition and presented for follow-up 3 years later with no disabilities.