Abstract

Acquired factor V inhibitor is a rare cause of factor V deficiency [1] with a clinical spectrum ranging from the absence of symptoms to life-threatening bleeding manifestations [2,3]. Inhibitors may develop as spontaneous autoantibodies or as alloantibodies after exposure to bovine thrombin preparations. There is no defined standard care. Immunosuppressive agents are the most commonly proposed management, or alternately supportive care with recombinant activated factor VII [1,4]. Extrapolating the management recommendations of factor VIII inhibitors, the use of rituximab has been recently suggested [5,6]. However, there is scarce information about the safety and long-term efficacy of this therapy. In addition, the rarity of the disorder limits the source of information to extensively studied individual cases or small series. Here, we report the results obtained during long-term follow-up of two cases of spontaneous factor V inhibitors managed with rituximab.