title: Spontaneous multilevel airway haemorrhage in acquired haemophilia A.
abstract: BACKGROUND
  Acquired haemophilia A is caused by the development of an autoantibody to factor VIII in a person with previously normal haemostasis. The most common clinical presentation in hereditary haemophilia is intra-articular bleeding. In contrast, acquired haemophilia more commonly presents with skin, soft tissue and mucosal haemorrhages.
  
  
  METHODS
  We present a case of a patient with idiopathic acquired haemophilia A, whose initial presentation was that of spontaneous submental and submandibular haematoma which rapidly progressed to involve multiple sites in the airway, ultimately necessitating an emergent tracheotomy. In view of the extremely rare occurrence of this phenomenon, a systematic review and summary of all previously reported cases is also presented.
  
  
  RESULTS
  The diagnostic and management dilemmas encountered in this case are presented from an otolaryngologist's perspective.
  
  
  CONCLUSIONS
  Otolaryngologists' awareness of underlying haematological pathologies and their natural course of disease in cases of severe airway haemorrhage is of paramount importance in anticipating evolving issues in management of these patients.
authors: Department of Otorhinolaryngology - Head & Neck Surgery, Changi General Hospital, Singapore, Singapore.
authors: Anna
authors: A
authors: See
authors: Department of Otorhinolaryngology - Head & Neck Surgery, Changi General Hospital, Singapore, Singapore.
authors: Siti Radhziah
authors: SR
authors: Sudirman
authors: Department of Otorhinolaryngology - Head & Neck Surgery, Changi General Hospital, Singapore, Singapore. hxy1976@yahoo.com.
authors: Xin Yong
authors: XY
authors: Huang
mesh_terms: D001779:Blood Coagulation Factors / Q000627:therapeutic use; D005169:Factor VIII; D006406:Hematoma / Q000000981:diagnostic imaging / Q000209:etiology*; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy; D006470:Hemorrhage / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012140:Respiratory Tract Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D014057:Tomography, X-Ray Computed
chemical_list: D001779:Blood Coagulation Factors; C078147:F8 protein, human; D005169:Factor VIII; C065655:anti-inhibitor coagulant complex
keywords: Airway; Haematoma; Haemophilia; Haemorrhage; Tracheostomy