A CONGENITAL VARIANT OF THROMBOTIC THROMBOCYTOPENIC PURPURA IN 2 SIBLINGS

We describe two siblings affected by chronic relapsing thrombotic thro mbocytopenic purpura from infancy. The elder brother, a 12-year-old bo g had 50 such episodes characterized by acute onset of fever, headache , drowsiness, vomiting, dark urine, thrombocytopenia and anemia. The y ounger sister, a 6-year-old girl, had 8 episodes with the same clinica l manifestations. Petechiae and ecchymoses on the estremities were pre sent throughout their lives. Furthermore, anemia with evidence of red blood cell fragmentation and thrombocytopenia were present chronically . Periodical transfusion of frozen fresh plasma prevented recurrent ep isodes. These cases suggest that there is a congenital variant of thro mbotic thrombocytopenic purpura.