Published online 1 April 2009
Haematologica, Vol 94, Issue 4, 566-575 doi:10.3324/haematol.2008.001743
Copyright © 2009 by Ferrata Storti Foundation
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Disorders of Hemostasis

International recommendations on the diagnosis and treatment of patients with acquired hemophilia A

Angela Huth-Kühne1, Francesco Baudo2, Peter Collins3, Jørgen Ingerslev4, Craig M. Kessler5, Hervé Lévesque6, Maria Eva Mingot Castellano7, Midori Shima8, Jean St-Louis9

1 SRH Kurpfalzkrankenhaus Heidelberg gGmbH and Hemophilia Center, Heidelberg, Germany
2 Thrombosis and Hemostasis Unit, Niguarda Hospital, Milan, Italy
3 Arthur Bloom Haemophilia Centre, University Hospital of Wales School of Medicine, Cardiff University, Cardiff, UK
4 Center for Hemophilia and Thrombosis, Skejby University Hospital, Department of Clinical Biochemistry, Aarhus, Denmark
5 Georgetown University Hospital, Lombardi Cancer Center, Division of Hematology/Oncology, Washington, DC, USA
6 Department of Internal Medicine, Centre Hospitalier Universitaire de Rouen-Boisguillaume, Rouen, France
7 Regional University Hospital Carlos Haya, Division of Hematology, Málaga, Spain
8 Department of Pediatrics, Nara Medical University, Nara, Japan
9 Hématologie-Oncologie, Hôpital Maisonneuve-Rosemont, Montréal, QC, Canada

Correspondence: Angela Huth-Kühne, SRH Kurpfalzkrankenhaus and Hemophilia Center Heidelberg gGmbH Bonhoefferstrasse 5, 69123 Heidelberg. E-mail:angela.huth-kuehne{at}kkh.srh.de

Acquired hemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies directed against circulating coagulation factor (F) VIII. Typically, patients with no prior history of a bleeding disorder present with spontaneous bleeding and an isolated prolonged aPTT. AHA may, however, present without any bleeding symptoms, therefore an isolated prolonged aPTT should always be investigated further irrespective of the clinical findings. Control of acute bleeding is the first priority, and we recommend first-line therapy with bypassing agents such as recombinant activated FVII or activated prothrombin complex concentrate. Once the diagnosis has been achieved, immediate autoantibody eradication to reduce subsequent bleeding risk should be performed. We recommend initial treatment with corticosteroids or combination therapy with corticosteroids and cyclophosphamide and suggest second-line therapy with rituximab if first-line therapy fails or is contraindicated. In contrast to congenital hemophilia, no comparative studies exist to support treatment recommendations for patients with AHA, therefore treatment guidance must rely on the expertise and clinical experience of specialists in the field. The aim of this document is to provide a set of international practice guidelines based on our collective clinical experience in treating patients with AHA and contribute to improved care for this patient group.

Key words: acquired hemophilia, bleeding, inhibitors, treatment, recommendations.


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