title: [Acquired haemophilia A: two cases].
abstract: BACKGROUND
  Acquired haemophilia A (AH) is an uncommon bleeding disorder that presents as multiple, disseminated spontaneous subcutaneous bleeds. Diagnosis may be made on the basis of prolonged activated partial thromboplastin time (aPTT). The severity of the disease is associated with the low risk of haemoglobin levels and with potential links with other diseases.
  
  
  METHODS
  Two men were hospitalized for extensive and spontaneous subcutaneous hematoma. In both cases, the International Normalized Ratio (INR) was normal, but aPTT was 3 times higher than normal. Autoantibodies against coagulation factor VIII confirmed the diagnosis of AH. The patients received immunomodulatory treatment. In one patient, diffuse large B-cell lymphoma was discovered one year after successful treatment of AH.
  
  
  CONCLUSIONS
  AH may be revealed by areas of bruising, subutaneous haematomas mimicking erythema nodosum, and muscle pain. APTT results alone can prompt the biologist to screen for factor VIII inhibitors. Aside from the risk of fatal bleeding, in half of all cases, the prognosis is determined by associated disorders such as blood dyscrasias, solid tumours, autoimmune diseases, use of certain medicines and pregnancy. After treatment for bleeding complications, therapy focuses on restoring the coagulation time. The aim of immunomodulatory therapy is to stem production of autoantibodies against coagulation factor VIII.
  
  
  CONCLUSIONS
  AH must be considered rapidly in order to reduce the risk of bleeding emergencies and to screen for potential related diseases.
authors.affiliation: Service de dermatologie, batiment Philippe-Canton, CHU, 6, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France. Electronic address: axelledevitry@yahoo.fr.
authors.forename: A
authors.initials: A
authors.lastname: de Vitry
authors.affiliation: Service de dermatologie, hôpital d'instruction des armées Legouest, 27, avenue de Plantières, 57070 Metz, France.
authors.forename: A
authors.initials: A
authors.lastname: Valois
authors.affiliation: Service de dermatologie, batiment Philippe-Canton, CHU, 6, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: M
authors.initials: M
authors.lastname: Weinborn
authors.affiliation: Service de dermatologie, hôpital d'instruction des armées Legouest, 27, avenue de Plantières, 57070 Metz, France.
authors.forename: A-L
authors.initials: AL
authors.lastname: Dupuy-de Fonclare
authors.affiliation: Service de dermatologie, batiment Philippe-Canton, CHU, 6, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: J-F
authors.initials: JF
authors.lastname: Cuny
authors.affiliation: Service de dermatologie, batiment Philippe-Canton, CHU, 6, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: A
authors.initials: A
authors.lastname: Barbaud
authors.affiliation: Service de dermatologie, batiment Philippe-Canton, CHU, 6, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: J-L
authors.initials: JL
authors.lastname: Schmutz
mesh_terms: D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D015897:Comorbidity; D004438:Ecchymosis / Q000209:etiology; D004844:Epistaxis / Q000209:etiology; D005169:Factor VIII / Q000276:immunology* / Q000502:physiology; D006467:Hemophilia A / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007680:Kidney Neoplasms / Q000150:complications* / Q000276:immunology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D011241:Prednisone / Q000627:therapeutic use; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D000069283:Rituximab; D005169:Factor VIII; D011241:Prednisone
keywords: Acquired haemophilia A; Anticorps anti-facteur VIII; Bleeds; Blood diseases; Bruises; Coagulation; Ecchymoses; Factor VIII antibody; Hématomes; Hémopathie; Hémophilie A acquise