Acquired @entity447 is a rare, but often severe @entity3459 caused by autoantibodies against @entity3459 factor, usually @entity2801 ( @entity2801 ). Between 1997 and 2004 we observed 14 @entity1 (mean age of 78 years) with acquired @entity447 . The aim of the present study was to investigate the effect of activated prothrombin complex concentrate (aPCC) for @entity548 and the response to @entity288 and @entity786 to eradicate the offending autoantibodies. The most common clinical presentations were severe profuse @entity2981 (12) and haematuria (5). Ten @entity1 were classified as idiopathic. At the time of diagnosis all @entity1 had a very low @entity2801 level, and one @entity1 also showed factor IX < 1%. High levels of antibodies to @entity2801 varying from 10 to 1340 Bethesda units (BU) and prolonged activated partial thromboplastin time were disclosed in all @entity1 . Eight severe @entity548 were treated with aPCC (FEIBA) at a dosage of 70 IU kg(-1) every 8 h until haemostasis. Ten @entity1 received @entity288 and @entity786 as immunomodulatory therapy. Effective haemostasis was achieved in all @entity548 after aPCC. Ten of 11 @entity1 responded either completely or partially to the immunomodulatory regime within 6 months. Five @entity1 achieved complete response (CR) whereas partial responses were seen in five patients. The anti- @entity2225 monoclonal antibody rituximab was given to two @entity1 in conventional doses and a CR was seen in one @entity1 . aPCC is effective in treating @entity548 in @entity1 with acquired @entity447 with high inhibitor levels. The combination of oral @entity288 and @entity786 seems to be effective to eradicate the inhibitor.

Acquired @entity447 : management of XXXX and immune therapy to eradicate autoantibodies.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity2801 :: ('2157', 'Gene') :: ['FVIII', 'factor VIII'] @entity548 :: ('MESH:D006470', 'Disease') :: ['acute bleeds', 'bleeds'] @entity447 :: ('MESH:D006467', 'Disease') :: ['haemophilia'] @entity2225 :: ('54474', 'Gene') :: ['CD20'] @entity786 :: ('MESH:D003520', 'Chemical') :: ['cyclophosphamide'] @entity3459 :: ('MESH:D025861', 'Disease') :: ['a coagulation', 'bleeding disorder'] @entity2981 :: ('MESH:D003288', 'Disease') :: ['bruising'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity548 :: (MESH:D006470,Disease) :: ['bleeds']
