Figure 1.
TTP as a function of age of onset and mechanism for ADAMTS13 deficiency. The proportions of adulthood-/childhood-onset TTP and acquired/inherited TTP, respectively, presented in this figure were calculated from the data of the French Registry for TTP (840 patients).10,13 These data are in agreement with miscellaneous demographic data reported in the literature by other teams.3,5-9 The diagram shows 100 patients with TTP, each patient being represented by a symbol, either a square for patients with adulthood-onset TTP (91%) or a circle for patients with childhood-onset TTP (9%). Acquired TTP is presented in blue (94.5%), and inherited TTP (USS) in red (5.5%). Interestingly, the proportion of USS is very low (2.5%) in adulthood-onset TTP, whereas it is as high as 33% in childhood-onset USS.