Diagnosis, laboratory aspects and management of acquired hemophilia A.
Acquired hemophilia A (AHA) is a rare autoimmune disease, characterized by severe, often life-threatening hemorrhages in patients without a prior history of bleeding disorder. It most frequently occurs in the elderly, and may be associated with other clinical conditions, such as cancer, autoimmune diseases, pregnancy or without a relevant cause. Diagnosis and correct therapy are crucial for the patient's outcome. Management of the disease consists of gaining immediate control of acute bleeding and the starting of an immunosuppressive therapy in order to eradicate the anti-factor VIII autoantibody. Factor VIII bypassing agents, such as prothrombin complex concentrates or recombinant activated factor VII, have proven effective in bleeding control, whereas the combined therapy of cyclophosphamide and corticosteroids seems to be, at present, the best immunosuppressive option. Other treatments including Rituximab, immunoadsorption or induction of immune tolerance are still experimental, and need to be validated through controlled clinical trials.
Toschi
Vincenzo
V
Servizio di Immunoematologia e Trasfusione, Centro Emostasi e Trombosi, AO Ospedale San Carlo Borromeo, Via Pio II, 3, 20153, Milan, Italy. vincenzo.toschi1@alice.it
Baudo
Francesco
F
D001327:Autoimmune Diseases / Q000175:diagnosis; D005169:Factor VIII / Q000032:analysis*; D006467:Hemophilia A / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans
D005169:Factor VIII
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