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Factor XI (FXI) deficiency (hemophilia C or Rosenthal disease) was first described in the 1950s in a multigenerational family experiencing bleeding related to surgery and dental procedures. Managing patients with FXI deficiency presents several challenges, including a lack of correlation of bleeding symptoms with FXI activity levels, the large volume of fresh frozen plasma required to achieve hemostatic FXI levels, lack of availability of FXI concentrate in certain regions of the world, and the inherent thrombotic risk associated with replacement therapy. This article summarizes presentation, diagnosis, and management of patients with FXI deficiency in a variety of clinical settings.

SummaryPlasma thromboplastin antecedent is a beta-globulin which is essential to normal coagulation. Its deficiency results in a hemorrhagic disease which is relatively less severe than either AHG or PTC deficiency. Transmitted as an autosomal dominant gene with variable expression and penetrance, the disease occurs in both males and females. The incidence of PTA deficiency seems to be high in comparison with the other hereditary clotting protein abnormalities. At present, our series numbers 39 cases (29 families). Since PZA is a catalytic, stable factor, which is present in serum, it is well corrected by both fresh or stored blood or plasma (15% replacement). The correction gradually disappears over the period of one week.