RT Journal Article
T1 International recommendations on the diagnosis and treatment of patients with acquired hemophilia A.
A1 Huth-Kühne, Angela
A1 Baudo, Francesco
A1 Collins, Peter
A1 Ingerslev, Jørgen
A1 Kessler, Craig M
A1 Lévesque, Hervé
A1 Mingot Castellano, Maria Eva
A1 Shima, Midori
A1 St-Louis, Jean
K1 Acquired hemophilia
K1 Bleeding
K1 Inhibitors
K1 Treatment
K1 Recommendations
K1 Hemofilia A
K1 Hemorragia
K1 Tiempo de tromboplastina parcial
K1 Tratamiento medicamentoso combinado
K1 Cooperación Internacional
AB 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.
PB Ferrata Storti Foundation
SN 0390-6078
YR 2009
FD 2009-04
LK http://hdl.handle.net/10668/1204
UL http://hdl.handle.net/10668/1204
LA en
NO Huth-Kühne A, Baudo F, Collins P, Ingerslev J, Kessler CM, Lévesque H, et al. International recommendations on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica. 2009; 94(4):566-75
NO Journal Article; Practice Guideline; Research Support, Non-U.S. Gov't;
DS RISalud
RD Apr 11, 2025