[Acquired haemophilia (acquired factor VIII inhibitor)].
Acquired haemophilia is a rare disorder. The clinical picture ranges from mild ecchymosis and anaemia to life threatening bleeding in up to 20% of patients. The disease is produced by an antibody against Factor VIII and it usually occurs in the elderly, with no previous history of a bleeding disorder. It can be associated to an underlying condition such as cancer, autoimmune disorders, drugs or pregnancy. It has a typical laboratory pattern with isolated prolonged activated partial thromboplastin time (aPTT) that fails to correct upon mixing tests with normal plasma and low levels of factor VIII. Treatment recommendations are based on controlling the acute bleeding episodes with either bypassing agent, recombinant activated factor VII or activated prothrombin complex concentrate, and eradication of the antibody with immunosuppressive therapy.
Servicio de Hematología y Hemoterapia, Hospital Británico de Buenos Aires, Argentina. E-mail: jceresetto@intramed.net.
José M
JM
Ceresetto
Cristina
C
Duboscq
Carlos
C
Fondevila
Miguel
M
Tezanos Pinto
D001323:Autoantibodies / Q000097:blood; D005169:Factor VIII / Q000032:analysis; D006467:Hemophilia A / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006470:Hemorrhage / Q000628:therapy; D006801:Humans
D001323:Autoantibodies; D005169:Factor VIII
acquired hemophilia; bleeding; factor VIII inhibitor