Figure 1.
Age distribution and ADAMTS13 inhibitor levels in acquired idiopathic (ai−) TTP with severe deficiency of ADAMTS13 activity.
Upper panel shows the age distribution of 186 patients with severe deficiency of ADAMTS13 activity under 5%. We found wide range of the age at TTP bouts from 8 months old to 87 years old. The highest incident peak was found around 60 years old. Lower panel shows the distribution of ADAMTS13 inhibitors in 186 ai-TTP patients with severe deficiency of ADAMTS13 activity. We found ADAMTS13 inhibitors (≥0.5 BU/ml) in 182 patients (97.8%). High titer inhibitors ≥2.0 BU/ml was seen in 101 patients (54.3%).
Table 1.
Clinical features in ai-TTP patients with severe deficiency of ADAMTS13:AC.
Table 2.
Comparison of our findings with those reported from Europe, Asia, and the United States for acquired idiopathic TTP patients with severely deficient ADAMTS13:AC levels.