Acquired Hemophilia A: A Potentially Fatal Bleeding Disorder.

Abstract

Acquired hemophilia A (AHA) is a rare autoimmune hematological disorder that has an incidence of about 1.5 cases per million people per year. It occurs in the elderly with the median age of 75 years, and most of the cases are idiopathic. It occurs due to the development of factor VIII inhibitor, which is an autoantibody against factor VIII leading to potentially life-threatening bleeding episodes. The diagnosis of AHA is often delayed and challenging. We report a case of an 86-year-old male who initially presented with signs and symptoms of a stroke. He was found to have oral mucosal bleeding and swelling of the floor of the mouth. He later developed epistaxis, hematuria, and melena. He had an isolated elevation of activated partial thromboplastin time (APTT) with very high levels of factor VIII inhibitor (1152 Bethesda units) and very low levels of Factor VIII (<1%). He was managed with supportive transfusion, bypass agents, and immunosuppressive therapy. AHA is a rare autoimmune bleeding disorder and is more commonly seen in the elderly population. Bleeding in AHA is usually sudden and sometimes life-threatening. Hence early hemostasis with bypassing agents and treatment with immunosuppressive agents should be initiated. Due to the rarity of the disorder, it is crucial to report AHA cases to create awareness and increase the index of suspicion of the clinicians for early diagnosis and treatment to prevent morbidity and mortality.