Clinical features in ai-TTP patients with severe deficiency of ADAMTS13:AC.

<p>NS: not significant difference (≥0.05).</p><p>Overall p values were caluculated using the Kruskal-Wallis H tests or chi-square tests with Yates' correction for 2×4 tables.</p><p>Significant differnces between 4 groups (overall p<0.05) were further analyzed by Mann-Whitney U-test or chi-squre test.</p>a<p>p<0.01 between Group 1 and Groups 2, 3, 4.</p>b<p>p<0.01 between Group 1 and Groups 3, 4, and between Group 2 and Groups 3, 4.</p>c<p>p<0.01 between Group 1 and Groups 3, 4.</p>d<p>p<0.05 between Group 2 and Group 4.</p

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.

<p> <b>ND: no data.</b></p><p> <b>Median (minimum-maximum).</b></p

Age distribution and ADAMTS13 inhibitor levels in acquired idiopathic (ai−) TTP with severe deficiency of ADAMTS13 activity.

<p>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%).</p