title: Acquired haemophilia A as a blood transfusion emergency.
abstract: BACKGROUND
  Acquired haemophilia is a rare autoimmune disorder caused by autoantibodies directed in the majority of the cases against clotting factor VIII. This disorder is characterised by the sudden onset of bleeding that not rarely may be life-threatening and need transfusion support. Most reports on this condition describe the need for blood transfusions during the acute, haemorrhagic phase, but the number of transfused red cell units is often unknown.
  
  
  METHODS
  In the last 5 years, 14 patients with acquired haemophilia A were identified in the transfusion and haemophilia centres of Verona and Castelfranco Veneto. The transfusion support for these 14 patients was analyzed in this retrospective survey.
  
  
  RESULTS
  The 14 patients required a total of 183 red cell units. The average transfusion requirement was 13 red cells units/patient, with a range from 0 to 38 units.
  
  
  CONCLUSIONS
  Eleven of the 14 patients studied needed strong transfusion support to enable any further management of the haemorrhages, as well as for eradication treatment of the autoantibodies to factor VIII. A relevant part of the management of haemorrhagic symptoms as well as the first choice for any further treatment (bleeding or the cure of the underlying disease) is transfusion of red blood cells.
authors.affiliation: Servizio Immunoematologia e Trasfusione, Centro Regionale per le Malattie del Sangue e Centro emofilia, ASL 8, Ospedale di Castelfranco Veneto, Castelfranco Veneto, Italy. giuseppe.tagariello@ulssasolo.ven.it
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Tagariello
authors.forename: Roberto
authors.initials: R
authors.lastname: Sartori
authors.forename: Paolo
authors.initials: P
authors.lastname: Radossi
authors.forename: Renzo
authors.initials: R
authors.lastname: Risato
authors.forename: Giovanni
authors.initials: G
authors.lastname: Roveroni
authors.forename: Cristina
authors.initials: C
authors.lastname: Tassinari
authors.forename: Annachiara
authors.initials: A
authors.lastname: Giuffrida
authors.forename: Giorgio
authors.initials: G
authors.lastname: Gandini
authors.forename: Massimo
authors.initials: M
authors.lastname: Franchini
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood* / Q000150:complications / Q000188:drug therapy; D001778:Blood Coagulation Disorders / Q000097:blood* / Q000188:drug therapy / Q000209:etiology; D003422:Critical Care; D017707:Erythrocyte Transfusion; D005169:Factor VIII / Q000276:immunology*; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use; D012189:Retrospective Studies
chemical_list: D001323:Autoantibodies; D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa