Huth-Kühne, AngelaBaudo, FrancescoCollins, PeterIngerslev, JørgenKessler, Craig MLévesque, HervéMingot Castellano, Maria EvaShima, MidoriSt-Louis, Jean2013-07-032013-07-032009-04Huth-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):566-750390-6078http://hdl.handle.net/10668/1204Journal Article; Practice Guideline; Research Support, Non-U.S. Gov't;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.enAcquired hemophiliaBleedingInhibitorsTreatmentRecommendationsHemofilia AHemorragiaTiempo de tromboplastina parcialTratamiento medicamentoso combinadoCooperación InternacionalMedical Subject Headings::Geographicals::Geographic Locations::Europe::GermanyMedical Subject Headings::Diseases::Hemic and Lymphatic Diseases::Hematologic Diseases::Blood Coagulation Disorders::Blood Coagulation Disorders, Inherited::Hemophilia AMedical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::HemorrhageMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::HumansMedical Subject Headings::Disciplines and Occupations::Social Sciences::Internationality::International CooperationMedical Subject Headings::Phenomena and Processes::Circulatory and Respiratory Physiological Phenomena::Blood Physiological Phenomena::Partial Thromboplastin TimeMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Therapeutics::Drug Therapy::Drug Therapy, Combinationresearch article19336751open access10.3324/haematol.2008.0017431592-8721PMC2663620