Acquired hemophilia A

Servizio di Immunoematologia e Trasfusione, Centro Emofilia, Azienda Ospedaliera di Verona, Verona, Italy.
Hematology (Impact Factor: 1.25). 05/2006; 11(2):119-25. DOI: 10.1080/10245330600574185
Source: PubMed


Acquired hemophilia A is a rare but severe autoimmune bleeding disorder, resulting from the presence of autoantibodies directed against clotting factor VIII. The etiology of the disorder remains obscure, although approximately half of all cases are associated with other underlying conditions. A prompt diagnosis and appropriate management enable effective control of this acquired hemorrhagic disorder: the aims of therapy are to terminate the acute bleeding episode and eliminate or reduce the inhibitor. The recent availability of bypassing agents, first activated prothrombin complex concentrates and then recombinant activated factor VII, has significantly reduced mortality during the acute phase of the disease in patients with high titer inhibitors. On another front, immunosuppressive therapy (corticosteroids and cytotoxic agents, alone or in various combinations) has resulted in long-term inhibitor suppression in up to 70% of the cases. Moreover, new therapeutic strategies (anti-CD20 monoclonal antibody and immune tolerance protocols) are very promising and may further improve the prognosis of acquired hemophilia A.

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    • "For this, provision of immunosuppressive therapy is critical. In some cases of postpartum and drug-induced acquired hemophilia that resolves spontaneously, immunosuppressive therapy may be unnecessary [69]. However, even if bleeding symptoms are mild, the risk of severe and fatal hemorrhage persists unless inhibitors are eradicated. "
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    ABSTRACT: Acquired hemophilia A (AHA) is a rare hemorrhagic disease in which autoantibodies against coagulation factor VIII- (FVIII-) neutralizing antibodies (inhibitors) impair the intrinsic coagulation system. As the inhibitors developed in AHA are autoantibodies, the disease may have an autoimmune cause and is often associated with autoimmune disease. Although acute hemorrhage associated with AHA may be fatal and is costly to treat, AHA is often unrecognized or misdiagnosed. AHA should thus be considered in the differential diagnosis particularly in postpartum women and the elderly with bleeding tendency or prolonged activated partial thromboplastin time. Cross-mixing tests and measurement of FVIII-binding antibodies are useful to confirm AHA diagnosis. For treatment of acute hemorrhage, hemostatic therapy with bypassing agents should be provided. Unlike in congenital hemophilia A with inhibitors, in which immune tolerance induction therapy using repetitive infusions of high-dose FVIII concentrates is effective for inhibitor eradication, immune tolerance induction therapy has shown poor efficacy in treating AHA. Immunosuppressive treatment should thus be initiated to eradicate inhibitors as soon as the diagnosis of AHA is confirmed.
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    ABSTRACT: INTRODUCTION: The objective of this work was to review current data about the pathophysiology, clinical features, and treatment of thrombotic microangiopathies. CURRENT KNOWLEDGE: Thrombotic microangiopathies are microvascular occlusive disorders characterized by systemic or intrarenal aggregation of platelets, thrombocytopenia, and mechanical injury to erythrocytes. In thrombotic thrombocytopenic purpura, systemic microvascular aggregation of platelets causes ischemia in the brain and other organs. In the hemolytic-uremic syndrome, platelet-fibrin thrombi occlude predominantly the renal circulation. Thrombotic microangiopathy is a rare disorder whose varied clinical manifestations result from the formation of platelet-rich thrombi within the microvasculature and consequent tissue ischemia. The clinical features are acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. This diagnosis is of considerable importance because of the possible fulminant clinical course. Some atypical forms may be unrecognized. Plasma exchange is the current reference treatment of thrombotic thrombocytopenic purpura. However, in the light of recent publications, either infusions of concentrates of purified enzyme or more intensive immunosuppressive therapy would be more specific.
    No preview · Article · May 2007 · Journal des Maladies Vasculaires
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    ABSTRACT: Nine unusual bleeding and clotting disorders (or mimickers of such) are described in the format of case presentations, with focus on clinical history, images and diagnostic tests, followed by a discussion of the disease itself and a summarizing clinical teaching point. The disease entities discussed are acquired factor VIII inhibitor, acquired von Willebrand factor inhibitor, haemophilic pseudotumour, Gardner-Diamond syndrome, coumarin-induced skin necrosis, purple toe syndrome, brachiocephalic vein thrombosis with breast enlargement, and leg swelling due to nephrogenic fibrosing dermopathy and lymphoedema. The publication is meant to demonstrate the fascination of clinical coagulation.
    Preview · Article · Sep 2007 · Hamostaseologie
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