TTP and HUS are two disorders with many similarities. Though their first de
scriptions appeared at different time in history, there has been a trend am
ong physicians to consider them as the same clinical entity. However, in re
cent years new research findings on the pathophysiology of TTP and HUS have
revealed some differences between the two disorders. In this paper, we wil
l review the current approaches to the clinical and laboratory diagnosis of
TTP and HUS, as well as therapeutic strategies. We will also summarize the
recent advances in three areas in the study of the pathophysiology of TTP
and HUS, namely the newly discovered von Willebrand factor multimer-cleavin
g protease, endothelial cell apoptosis induced by serum from patients with
TTP and atypical HUS and the activation of complement system. Since disting
uishing and differentiating between TTP and HUS may help to develop more ef
fective therapies targeted at key steps of the disease development, we will
discuss possible ways of reclassifying the TTP-HUS disorders. In the end,
we also present our views on possible future development.