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Acquired haemophilia in a patient with discoid lupus



Ann Hematol (2011) 90:119–120 DOI 10.1007/s00277-010-0972-7 LETTER TO THE EDITOR Iris Appelmann & Christoph Biermann & Inken Hartig & Wolfgang E. Berdel & Rolf Mesters Received: 14 December 2009 / Accepted: 19 April 2010 / Published online: 14 May 2010 # Springer-Verlag 2010 Dear Editor, Acquired haemophilia A is a rare but potentially lifethreatening bleeding disorder caused by autoantibodies against the coagulation factor (F) VIII (FVIII inhibitor) which is in some cases associated with autoimmune diseases, solid tumours, haemotologic malignancies or can occur in the post-partum period. For further information concerning the pathophysiology of acquired haemophilia, please refer to [1]. Mortality is rather high in newly diagnosed acquired haemophilia ranging from 8% to 22% in different studies [2–5]. A major cause of significant mortality and morbidity is the frequent confusion with other life-threatening conditions such as disseminated intravascular coagulation, thus often leading to uncontrolled bleeding prior to correct diagnosis of the disease. Effective treatment involves not only the initial treatment of acute haemorrhage with factor VIII concentrates, a factor-VIII-bypassing-agent such as recombinant FVIIa or activated prothrombin complex concentrate but also the eradication of the inhibitor by immunosuppression, plasmapheresis or immunoadsorption or a combination of these modalities [1, 5,



Annals of HematologySpringer Journals

Published: Jan 1, 2011

DOI: 10.1007/s00277-010-0972-7

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