Acquired Hemophilia A: Rare Cause of Upper Airway Hematoma and a Literature Review

Abstract

Background: Acquired hemophilia A (AHA) is a rare blood disorder with high morbidity and even mortality as severe bleeding can occur in up to 90% of affected patients. Unlike congenital hemophilia which presents with intra-articular bleeding, acquired hemophilia causes bleeding into the skin, muscle, mucous membranes and soft tissues.

Case presentation: We report an unusual case of upper airway hematoma in a 61-year-old man who presented with acute onset dysphagia and shortness of breath. There were bruises on his neck and blood clots on the floor of mouth when he was examined. Endoscopic examination revealed an extensive laryngeal hematoma. A prolonged activated partial thromboplastin time (APTT) prompted us to investigate for factor VIII deficiency leading to the diagnosis of AHA. He recovered completely after a treatment regime instituted by the hematology team without suffering any grave debilitating events.

Conclusion: AHA with laryngeal hematoma is a rare condition with only a handful of cases reported. Although a life-threatening disease, it is easily reversed with early recognition and administration of medical therapy involving the hematology team.

Keywords: Acquired hemophilia A; Factor VIII deficiency; Laryngeal hematoma; Larynx.

Fig. 1

(a)Bruises noted over the neck. (b & c) Hematoma over the right pyriform fossa, aryepiglottic fold, arytenoid, false cord, vallecula and lingual surface of epiglottis

Fig. 2

(a & b) Endoscopic view of the larynx after one month of treatment showing resolved hematoma