Sammanfattning
Acquired haemophilia is a rare bleeding disorder with high morbidity. The incidence is approximately 1.5 per million and year. In the Nordic countries the incidence is only 0.7, suggesting a high unrecorded number of cases. A specific coagulation assessment is necessary for adequate treatment in acute bleeding events. Immune suppressive therapy should be instituted as soon as the diagnosis is established with Prednisolone 1 mg per kg body weight with or without cytotoxic drugs during 4-6 weeks to eliminate the activity of the autoantibodies. If no signs of remission after 6-8 weeks a second line of immune suppression therapy should be considered e.g. with rituximab.