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Acquired hemophilia A: A rare cause of bleeding in the Intensive Care Unit. A case report and review of the literature [Greek]
Authors: Soulountsi V, Karachristos Ch, Schizodimos Th, Mouskeftara I, Lavrentieva A
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Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies to coagulation factor VIII (FVIII) leading to potentially severe bleeding diathesis that carries a high rate of morbidity and mortality. It should be suspected in patients with unexplained prolonged aPTT, normal PT and acute abnormal bleedings without personal or familiar history of congenital bleeding disorders. The early diagnosis of AHA is difficult since the symptoms of AHA are different from those of congenital hemophilia as well as there is a low index of clinical suspicion because of the contemporary use of anticoagulants in the elderly, where it usually manifests. AHA has a bimodal distribution with first distribution peak between 20-30 years and the second major peak in elderly patients over 60 years of age. Common manifestations of AHA are bleeding from skin, soft tissues and mucosal (e.g., epistaxis, gastrointestinal and urological bleeding), retroperitoneal hematomas, postpartum hemorrhage while sometimes the laboratory disorder can be recognized without clinical symptoms of bleeding. ΑΗΑ can occur in people without or with an underlying disease (autoimmune, malignancy, diabetes, drugs) and in pregnancy. To establish the diagnosis, it is important to follow the diagnostic algorithm proposed by the guidelines. Management of AHA includes treatment of the underlying condition that caused the disorder (if it is recognized and is treatable), treatment of acute bleeding episodes, and prevention of bleeding by eradication the autoantibody. For this reason, clinicians should optimize hemostatic and immunosuppressive therapy and the therapy should be started as soon as the diagnosis is established. Regarding hemostatic therapy, the most widely used agents are recombinant factor VII and activated prothrombin complex concentrate. Regarding immunosuppressive therapy, first line therapies are corticosteroids alone or in combination with cyclophosphamide and rituximab based on patients’ characteristics (FVIII activity, inhibitor titer) and patients’ response to previous treatment. High mortality of AHA is due to bleeding during the first days after diagnosis or to infections, either as a consequence of the immunosuppressive treatment applied to eradicate the autoantibody either of the patients’ comorbidities. Relapse rate upon discontinuation of immunosuppressive therapy is 7-20% with a mean time of onset of 3-7.5 months. There are no recommendations regarding the use of immunosuppressive therapy in relapses.