Acquired Inhibitors of Coagulation Factors: Part I—Acquired Hemophilia A

Antonio Coppola; Emmanuel J. Favaloro; Antonella Tufano; Matteo N.D. Di Minno; Anna Maria Cerbone; Massimo Franchini

doi:10.1055/s-0032-1315757

Seminars in Thrombosis and Hemostasis, Inhaltsverzeichnis

Semin Thromb Hemost 2012; 38(05): 433-446
DOI: 10.1055/s-0032-1315757

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Acquired Inhibitors of Coagulation Factors: Part I—Acquired Hemophilia A

Autoren

Antonio Coppola

¹Department of Clinical and Experimental Medicine, Regional Reference Center for Coagulation Disorders, Federico II University Hospital, Naples, Italy
Emmanuel J. Favaloro

²Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Westmead, Australia
Antonella Tufano

¹Department of Clinical and Experimental Medicine, Regional Reference Center for Coagulation Disorders, Federico II University Hospital, Naples, Italy
Matteo N.D. Di Minno

¹Department of Clinical and Experimental Medicine, Regional Reference Center for Coagulation Disorders, Federico II University Hospital, Naples, Italy
Anna Maria Cerbone

¹Department of Clinical and Experimental Medicine, Regional Reference Center for Coagulation Disorders, Federico II University Hospital, Naples, Italy
Massimo Franchini

³Department of Transfusion Medicine and Hematology, C. Poma Hospital, Mantova, Italy

Abstract

Acquired hemophilia A (AHA) is a rare, but often severe, bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII). AHA occurs more frequently in the elderly and in association with several conditions, such as malignancies, autoimmune diseases, postpartum, or drug exposure; however, about half of the cases remain idiopathic. At variance with congenital hemophilia, where hemarthroses are the most common bleeding symptoms, hemorrhages in AHA involving soft tissues (muscle, skin) are more frequently reported. AHA is diagnosed in patients: with negative personal or family bleeding history; in which prolonged activated partial thromboplastin time is not corrected after mixing and incubating equal volumes of patient and normal plasma for ~2 hours at 37°C; FVIII levels are reduced; and a specific FVIII-inhibiting activity is detected. Prompt recognition and treatment of AHA are mandatory, as inadequate management and complications of the disease are associated with high mortality rates. Therapeutic approaches should aim to control acute bleeds, eradicate FVIII-autoantibody production, treat associated diseases, and when possible, eliminate them. Present knowledge about this often overlooked and challenging condition has significantly increased following establishment of recent national and international studies, as will also be reviewed in this article.

Keywords

acquired hemophilia A - autoantibodies - bleeding - inhibitors

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