Consensus recommendations for the diagnosis and treatment of acquired hemophilia A
1 Arthur Bloom Hemophilia Centre, School of Medicine, Cardiff University, University Hospital of Wales, Cardiff, CF14 4XN, UK
2 Thrombosis and Hemostasis Unit, Niguarda Hospital, I-20162 Milan, Italy
3 SRH Kurpfalzkrankenhaus Heidelberg GmbH and Hemophilia Centre, D-69123 Heidelberg, Germany
4 Centre for Hemophilia and Thrombosis, Skejby University Hospital, Department of Clinical Biochemistry, DK-8200 Aarhus, Denmark
5 Department of Medicine and Pathology, Division of Hematology/Oncology and Georgetown University Hospital, Lombardi Cancer Center, Division of Hem/Onc, Washington, DC 20057, USA
6 Regional Universitary Hospital Carlos Haya, Division of Hematology, E-29004 Málaga, Spain
7 Department of Pediatrics, Nara Medical University, 634-8522 Nara, Japan
8 Department of Medicine, Université de Montréal and Hématologie-Oncologie, Hôpital Maisonneuve-Rosemont, Montréal, QC H1T 2 M4, Canada
9 Department of Internal Medicine, Centre Hospitalier Universitaire de Rouen-Boisguillaume, F- 76031 Rouen, France
BMC Research Notes 2010, 3:161 doi:10.1186/1756-0500-3-161Published: 7 June 2010
Acquired hemophilia A (AHA) is a rare bleeding disorder caused by an autoantibody to coagulation factor (F) VIII. It is characterized by soft tissue bleeding in patients without a personal or family history of bleeding. Bleeding is variable, ranging from acute, life-threatening hemorrhage, with 9-22% mortality, to mild bleeding that requires no treatment. AHA usually presents to clinicians without prior experience of the disease, therefore diagnosis is frequently delayed and bleeds under treated.
Structured literature searches were used to support expert opinion in the development of recommendations for the management of patients with AHA.
Immediate consultation with a hemophilia center experienced in the management of inhibitors is essential to ensure accurate diagnosis and appropriate treatment. The laboratory finding of prolonged activated partial thromboplastin time with normal prothrombin time is typical of AHA, and the diagnosis should be considered even in the absence of bleeding. The FVIII level and autoantibody titer are not reliable predictors of bleeding risk or response to treatment. Most patients with AHA are elderly; comorbidities and underlying conditions found in 50% of patients often influence the clinical picture. Initial treatment involves the control of acute bleeding with bypassing agents. Immunosuppressive treatment to eradicate the FVIII inhibitor should be started as soon as the diagnosis is confirmed to reduce the time the patient is at risk of bleeding.
These recommendations aim to increase awareness of this disorder among clinicians in a wide range of specialties and provide practical advice on diagnosis and treatment.