Table 1. Clinical features in ai-TTP patients with severe deficiency of ADAMTS13:AC.

	All patients	Groups according to age
		1	2	3	4	Overall p
Age (years)	54 (37, 65)	<20	20∼<40	40∼<60	60∼
	Median (25, 75 percentile)
Patient Number	186	17	36	63	70
Female (%)	54.8	52.9	69.4	57.1	45.7	NS
“Pentad”
(1) Platelet count (×109/L), Median (25, 75 percentile)	10 (7, 16)	9 (7, 12)	10 (7, 20)	10 (6, 18)	10 (8, 15)	NS
(2) Hemoglobin (g/dL), Median (25, 75 pecentile)	7.3 (6.1, 8.7)	7.4 (5.4, 8.7)	6.7 (5.9, 7.8)	7.1 (6.0, 8.8)	7.8 (6.6, 8.8)	NS
(3) Renal involvement (%)	75.8	52.9	72.2	81.0	78.5	NS
Serum creatinine (mg/dL), Median (25, 75 percntile)	0.9 (0.7, 1.3)	0.58 (0.31, 0.80)	0.86 (0.70, 1.16)	0.95 (0.80, 1.50)	1.00 (0.80, 1.40)	<0.01a
Blood urea nitrogen (mg/dL), Median (25, 75 percntile)	24 (17, 37)	15 (12, 23)	19 (14, 26)	27 (17, 41)	27 (21, 43)	<0.01b
(4) CNS involvement (%)	79.0	47.0	69.4	82.5	88.6	<0.01c
(5) Fever (≥37.0°C) (%)	71.5	76.5	63.9	69.8	75.7	NS
Mortality in the current episode of TTP bouts (%)	16.1	5.9	5.6	11.1	28.6	<0.05d

NS: not significant difference (≥0.05).

Overall p values were caluculated using the Kruskal-Wallis H tests or chi-square tests with Yates' correction for 2×4 tables.

Significant differnces between 4 groups (overall p<0.05) were further analyzed by Mann-Whitney U-test or chi-squre test.

^ap<0.01 between Group 1 and Groups 2, 3, 4.

^bp<0.01 between Group 1 and Groups 3, 4, and between Group 2 and Groups 3, 4.

^cp<0.01 between Group 1 and Groups 3, 4.

^dp<0.05 between Group 2 and Group 4.