A Severely Burned Hemophiliac

Patients suffering from moderate or severe hemophilia A are particularly vulnerable to trauma injury, being on high risk of immediate exsanguination. Due to a rareness of this disease, there are very few reports about the management of severe injuries of the affected patients. No guidelines for the management of burn trauma of hemophiliac patients have been yet established. Since, to our knowledge, this is the first case report about a successful treatment of a severely burned hemophiliac, requiring intensive care, long-time intubation, and multiple epifascial necrosectomies of third grade wounds, the authors are proposing the approach to this condition. The factor VIII has been substituted, enhancing its activity to over 70% during the planned interventions and keeping it above 30% in the intervals between the operations. To minimize the risk of surgical bleeding, our therapeutic strategy consisted of multiple short radical operations. The patient was healed and was dismissed after 68 days. The surgical blood loss was comparable with noncoagulopathic patients with similar burn extension. In case of a vast burn injury of the moderate or severe hemophiliacs, where the trauma-related consumption coagulopathy is expected and deep necrosectomies are required, the authors suggest starting a substitution of the factor VIII immediately after the admission, followed by a structured operative planning.