UC Irvine

Thrombotic thrombocytopenic purpura (TTP) was originally described by Moschkowitz in a 16-year-old girl who presented with fever, anemia, central nervous system impairment, renal dysfunction, and respiratory and cardiac failure in 1924 [1]. In this patient, the pathologic finding of hyaline thrombi in the terminal arterioles of the majority organs was considered to be characteristic of the disorder. Over the years, after further clinical observation, the classical triad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and fluctuating central nervous system abnormalities was considered to be the gold standard for this diagnosis [2]. In addition, many of these patients present with fever and renal function impairment. Accordingly these additional features included in the triad have born out the concept of pentad for the diagnosis of TTP [3].