Acquired hemophilia a successfully treated with rituximab.
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.
Hematology and Stem Cell Transplantation Unit, IRCCS Centro di Riferimento Oncologico della Basilicata, Rionero in Vulture, Italy.
Giovanni
G
D'Arena
Hemostasis and Thrombosis Unit, IRCCS "Casa Sollievo della Sofferenza" Hospital, San Giovanni Rotondo, Italy.
Elvira
E
Grandone
Department of Clinical Medicine and Surgery, Regional reference Center for Coagulation Disorders, Federico II University, Naples, Italy ; Unit of cell and molecular biology in cardiovascular diseases, Centro Cardiologico Monzino, IRCCS, Milan, Italy.
Matteo Nicola Dario
MN
Di Minno
Scientific Direction, IRCCS Centro di Riferimento Oncologico della Basilicata, Rionero in Vulture, Italy.
Pellegrino
P
Musto
Department of Clinical Medicine and Surgery, Regional reference Center for Coagulation Disorders, Federico II University, Naples, Italy.
Giovanni
G
Di Minno