Successful self‐infusion of activated prothrombin complex concentrate for prophylaxis in a child with a factor VIII inhibitor

Regular self‐infusion of an activated prothrombin complex concentrate (APCC) has been successfully introduced to a 14‐year‐old boy with hemophilia A. The child was diagnosed as a neonate, and at age 7 years, developed a high titer (127 BU/mL) factor VIII inhibitor coincident with a protracted ankle joint bleeding. From age 7–10 years, he received on‐demand therapy using a prothrombin complex concentrate (PCC), PROPLEX‐ST. From age 10–14 years, he received prophylaxis with PROPLEX‐ST, initiated after an intracranial hemorrhage and coincident anamnestic inhibitor response. Throughout 7‐year period of PCC treatment, he experienced recurrent bleeding episodes. Self‐prophylaxis with APCC, FEIBA VH [Anti‐inhibitor Coagulant Complex] (50 U/kg/dose three times per week) using infusion pump was initiated at 14 years of age and has continued for 2 years. There were no bleeding, thrombotic events or other adverse events after initiation of this prophylaxis, and inhibitor levels decreased to 1 BU/mL. His quality of life was improved, particularly with respect to school. Our long observation proposes a well‐disciplined home‐based FEIBA prophylaxis in inhibitor‐positive hemophiliacs. Am. J. Hematol., 2006. © 2006 Wiley‐Liss, Inc.