Sociedad Argentina de Hematología

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Revista Argentina de Hematología


Volumen:    21    # Number : 1

Publication Date :    Enero - Abril    Year:    2017


Update in the therapeutic approach of Acquired FVIII Inhibitor (Acquired Hemophilia)

Authors: Quarchioni MA

Abstract: Acquired haemophilia is an auto-immune disease caused by an inhibitory antibody to factor VIII. The pattern of bleeding varies but patients remain at risk of life threatening bleeding until the inhibitor has been eradicated. The cornerstones of management are; rapid and accurate diagnosis, control of bleeding, investigation for an underlying cause and eradication of the inhibitor by immunosuppression. Patients should always be managed jointly with a specialist centre even if they present without significant bleeding. Despite an extensive literature, few controlled data are available and treatment guidelines are based on expert opinion. To treat bleeds recombinant factor VIIa and activated prothrombin complex concentrate are equally efficacious but both are superior to factor VIII or desmopressin. Immunosuppression should be started as soon as the diagnosis is made. Commonly used regimens are steroids alone or combined with cytotoxic agents. Rituximab is another recommended therapeutic option in the second line

Key words: Acquired hemophilia, inhibitor, bypass agents, FVIII, immunosuppression.

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