Surgical management of endometriosis in a severe Hemophilia A female patient and the role of transfusion medicine specialist: A case report with review of literature

Abstract

Hemophilia A is an X-linked recessive bleeding disorder occurs due to deficiency of factor VIII (FVIII). The disease manifests exclusively in males though it rarely occurs in females due to complex pathophysiological mechanisms. We present a rare case of female hemophilia due to skewed X-inactivation which adversely affected the quality of patient life. She presented with recurrent abdominal pain and was diagnosed with severe endometriosis and underwent total abdominal hysterectomy with left salpingo-oophorectomy and appendicectomy. She was infused recombinant factor VIII both prophylactically and postoperatively as per the World Federation of Hemophilia guidelines. Recombinant Factor VIII was supplemented every 12th hourly and Factor VIII activity levels were monitored daily. She was discharged uneventfully on the postoperative day 21 after screened negative for acquired inhibitors.

Keywords

• hemophilia a
• inherited bleeding disorder
• recombinant factor viii
• skewed x-inactivation