title: A Hematological Menace: Multiple Venous Thrombosis Complicated by Acquired Factor VIII Deficiency.
abstract: BACKGROUND
  Acquired hemophilia A (AHA) classically presents with spontaneous bleeding of mucosal sites, GI tract, and subcutaneous tissues, often leading to large hematomas and ecchymosis. Among documented cases, 50% are idiopathic and few have been associated with trauma or surgery. We present a case of life-threatening bleeding caused by AHA, following trauma and complicated by multiple venous thrombi.
  
  
  METHODS
  A 21-year-old man presented with multiple injuries secondary to trauma leading to extensive life-saving surgery. Two weeks post-operatively, he developed multiple deep venous thrombi and was started on anticoagulation. Twenty-four days post-operatively, he started bleeding from multiple mucosal sites and developed an abdominal hematoma. Anticoagulation was stopped, with administration of fresh frozen plasma and vitamin K. Diagnosis of AHA was made based on low factor VIII level and presence of factor VIII inhibitors after an appropriate battery of tests ruled out other possible diagnoses. He was started on steroids and recombinant factor VIIa, leading to immediate improvement. Once stable, Rituximab infusions resulted in decreasing factor VIII inhibitor levels, with gradual normalization of PTT.
  
  
  CONCLUSIONS
  AHA remains a diagnostic challenge because of its rarity, leading to delay in diagnosis and causing significant morbidity and mortality. Elevated PTT relative to PT/INR is a strong clue which should be followed by mixing studies. Very few cases have been associated with surgery or trauma and relatively few large, controlled trials have compared different treatment modalities for AHA. Growing evidence supports anti-CD20 (Rituximab) as an effective treatment option, as in this case.
authors.affiliation: Department of Internal Medicine, University of Kentucky, Lexington, KY, USA.
authors.forename: Robin
authors.initials: R
authors.lastname: Paudel
authors.affiliation: Department of Internal Medicine, Jersey City Medical Center, Jersey City, NJ, USA.
authors.forename: Luis W
authors.initials: LW
authors.lastname: Dominguez
authors.affiliation: Department of Internal Medicine, University of Kentucky, Lexington, KY, USA.
authors.forename: Prerna
authors.initials: P
authors.lastname: Dogra
authors.affiliation: Department of Internal Medicine, University of Kentucky, Lexington, KY, USA.
authors.forename: Saurav
authors.initials: S
authors.lastname: Suman
authors.affiliation: Department of Medicine, Jersey City Medical Center, Jersey City, NJ, USA.
authors.forename: Simon
authors.initials: S
authors.lastname: Badin
authors.affiliation: Department of Medicine, Jersey City Medical Center, Jersey City, NJ, USA.
authors.forename: Carrie
authors.initials: C
authors.lastname: Wasserman
mesh_terms: D015942:Factor VIIa / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006467:Hemophilia A / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D009104:Multiple Trauma / Q000601:surgery; D011183:Postoperative Complications; D011994:Recombinant Proteins / Q000627:therapeutic use; D000069283:Rituximab / Q000627:therapeutic use; D020246:Venous Thrombosis / Q000209:etiology* / Q000628:therapy; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007155:Immunologic Factors; D011994:Recombinant Proteins; D000069283:Rituximab; C103587:recombinant FVIIa; D015942:Factor VIIa