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Acquired Haemophilia: An Update

Aktuelle Rheumatologie, ISSN: 1438-9940, Vol: 47, Issue: 6, Page: 502-505
2022
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Article Description

Acquired haemophilia A is the most common form of inhibitor-induced aquired haemophilia. It can occur idiopathically, in association with other diseases or post partum. In the majority of cases, there are pronounced bleeding signs that can be life-Threatening. Important differential diagnoses are mentioned. The diagnosis is made on the basis of prolonged aPTT by plasma mixing tests and the Bethesda test. As treatment, local measures, coagulation factor preparations and tranexamic acid are used to stop bleeding, and immunosuppression with glucocorticoids, rituximab and cyclophosphamide to eliminate the inhibitory autoantibody. Further immunosuppressive treatment options to treat refractory cases are mentioned. Prognosis seems to depend mainly on the extent to which factor VIII is initially reduced.

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