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Acquired hemophilia A (AHA) is a rare bleeding disorder due to the development of specific autoantibodies against factor VIII. The anti-CD20 monoclonal antibody Rituximab has been proven to be effective in obtaining a long-term suppression of inhibitors of AHA, besides other immunosuppressive standard treatments. Here we describe a case of idiopathic AHA in a 60-year old man successfully treated with rituximab. He showed a complete clinical response with a normalization of clotting parameters after 5 weekly courses of rituximab given at a dose of 375 mg/sqm. , but after stopping rituximab, an initial worsening of coagulation parameters induced the addition of 3 further courses. At present, the patient is in complete clinical and hematological remission after 200 days. This case confirms that Rituximab may be a safe and useful tool to treat AHA and, a prolonged administration can overcome the initial resistance. However, the precise position of this drug in the therapeutic strategy (first or second-line, alone or in combination with other drugs) remains to be established and warrants further investigation.
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2) Gurcan HM, Keskin DB, Stern JNH, Nitzberg MA, Shekhani H, Ahmed AR. A review of the current use of rituximab in autoimmune diseases. Int Immunopharmacol 2009; 9: 10-25.
3) Ma AD, Carrizosa D. Acquired factor VIII inhibitors: pathophysiology and treatment. Hematol Am Soc Hematol Educ Program 2006; 432-7.
4) Collins PW. Therapeutic challenges in acquired factor VIII deficiency. Hematol Am Soc Hematol Educ Program 2012; 369-374
5) Baudo F, Collins P, Huth-Kuhne A, Levesque H, Marco P, Nemes L, Pellegrini F, Tengborn L, Knoebl P. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. Blood 2012; 120: 39-46.
6) D’Arena G, Grandone E, Di Minno MND, Musto P, Di Minno G. Rituximab to treat adult acquired hemofilia A. Ann Hematol (submitted)
7) Franchini M, Mannucci PM. Inhibitor eradication with rituximab in haemophilia: where do we stand? Br J Haematol 2014; prepublishe online.