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Title: International recommendations on the diagnosis and treatment of patients with acquired hemophilia A.
Authors: Huth-Kühne, Angela
Baudo, Francesco
Collins, Peter
Ingerslev, Jørgen
Kessler, Craig M
Lévesque, Hervé
Mingot Castellano, Maria Eva
Shima, Midori
St-Louis, Jean
metadata.dc.contributor.authoraffiliation: [Huth-Kühne ,A] SRH Kurpfalzkrankenhaus Heidelberg gGmbH and Hemophilia Center, Heidelberg, Germany. [Baudo,F] Thrombosis and Hemostasis Unit, Niguarda Hospital, Milan, Italy. [Collins,P] Arthur Bloom Haemophilia Centre, University Hospital of Wales School of Medicine, Cardiff University, Cardiff, UK. [Ingerslev,J] Center for Hemophilia and Thrombosis, Skejby University Hospital, Department of Clinical Biochemistry, Aarhus, Denmark. [Kessler,CM] Georgetown University Hospital, Lombardi Cancer Center, Division of Hematology/Oncology, Washington, DC, USA. [Lévesque,H] Department of Internal Medicine, Centre Hospitalier Universitaire de Rouen-Boisguillaume, Rouen, France. [Mingot Castellano,EM] Regional University Hospital Carlos Haya, Division of Hematology, Málaga, Spain. [Shima,M] Department of Pediatrics, Nara Medical University, Nara, Japan. [St-Louis,J] Hématologie-Oncologie, Hôpital Maisonneuve-Rosemont, Montréal, QC, Canada.
Keywords: Acquired hemophilia;Bleeding;Inhibitors;Treatment;Recommendations;Hemofilia A;Hemorragia;Tiempo de tromboplastina parcial;Tratamiento medicamentoso combinado;Cooperación Internacional
metadata.dc.subject.mesh: Medical Subject Headings::Geographicals::Geographic Locations::Europe::Germany
Medical Subject Headings::Diseases::Hemic and Lymphatic Diseases::Hematologic Diseases::Blood Coagulation Disorders::Blood Coagulation Disorders, Inherited::Hemophilia A
Medical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Hemorrhage
Medical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans
Medical Subject Headings::Disciplines and Occupations::Social Sciences::Internationality::International Cooperation
Medical Subject Headings::Phenomena and Processes::Circulatory and Respiratory Physiological Phenomena::Blood Physiological Phenomena::Partial Thromboplastin Time
Medical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Therapeutics::Drug Therapy::Drug Therapy, Combination
Issue Date: Apr-2009
Publisher: Ferrata Storti Foundation
Citation: 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
Abstract: 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.
Description: Journal Article; Practice Guideline; Research Support, Non-U.S. Gov't;
metadata.dc.identifier.doi: 10.3324/haematol.2008.001743
ISSN: 1592-8721 (Online)
0390-6078 (Print)
Appears in Collections:01- Artículos - Hospital Regional de Málaga

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