Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare pediatric disease, yet has grave outcome if not managed properly. It shares many common clinical features with hemolytic uremic syndrome (HUS). While it is still disputable whether TTP and HUS are clinically related disorders, recent studies indicate that severe deficiency of a von Willebrand factor (vWF) cleaving protease is one of the primary causes of TTP. In this discussion, we will focus on TTP in reference to the deficiency of this protease, with the objective of increasing awareness among pediatric healthcare professionals of this less commonly recognized disease.

Keywords: Thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, TTP, HUS.