Table 1 Clinical characteristics, plasma... | American Society of Hematology

Table 1

Clinical characteristics, plasma ADAMTS13 activity, inhibitors of TTP patients, and sensitivity of WT ADAMTS13 and novel variants to the inhibition by patient plasma

Patient no.	Age, y	Sex	Platelet count, × 10^9/L	Hemato-crit, %	LDH, U/L	Creatinine, mg/dL	CNS, signs and symptoms	ADAMTS13 activity by rF-vWF73, %	Plasma anti-ADAMTS13 inhibitors	Plasma anti-ADAMTS13IgG, U/mL	Sensitivity to patient plasma inhibition
Patient no.	Age, y	Sex	Platelet count, × 10^9/L	Hemato-crit, %	LDH, U/L	Creatinine, mg/dL	CNS, signs and symptoms	ADAMTS13 activity by rF-vWF73, %	Plasma anti-ADAMTS13 inhibitors	Plasma anti-ADAMTS13IgG, U/mL	WT	M1	M2	M3	M4	M5
1	44	Female	15	23	695	1.1	Yes	< 5	+	84.2	++	++	++	+	−	−
2	56	Female	19	29	654	1.1	Yes	< 5	+	35.4	++	++	++	+	+	+
3	45	Female	88	28	995	0.7	Yes	< 5	+	81.9	++	++	++	−	−	−
4	52	Female	52	20	866	1.1	Yes	< 5	+	132.0	++	++	++	−	−	−
5	79	Female	7	27	1660	1.4	Yes	< 5	+	118.0	++	++	++	+	−	−
6	34	Male	15	23	2703	0.9	Yes	< 5	+	127.5	++	++	++	+	−	−
7	34	Male	0	33	859	1.9	No	< 5	+	162.0	++	++	++	−	−	−
8	23	Female	11	18	3412	0.8	Yes	< 5	+	132.0	++	++	++	−	−	−
9	42	Female	23	17	3259	0.8	Yes	< 5	+	168.0	++	++	++	−	−	−
10	21	Female	9	13	1489	1.5	Yes	< 5	+	72.0	++	++	++	+	+	+
11	61	Female	33	28	511	0.8	Yes	< 5	+	221.0	++	++	++	−	−	−
12	42	Male	16	24	6517	1.2	No	< 5	+	147.2	++	++	++	+	−	−
N = 12	43*		15.5*	23.5*	1242*	1.1*	(83.3)	(100)	(100)	(100)	(100)	(100)	(100)	(50)	(17)	(17)

Patient no.	Age, y	Sex	Platelet count, × 10^9/L	Hemato-crit, %	LDH, U/L	Creatinine, mg/dL	CNS, signs and symptoms	ADAMTS13 activity by rF-vWF73, %	Plasma anti-ADAMTS13 inhibitors	Plasma anti-ADAMTS13IgG, U/mL	Sensitivity to patient plasma inhibition
Patient no.	Age, y	Sex	Platelet count, × 10^9/L	Hemato-crit, %	LDH, U/L	Creatinine, mg/dL	CNS, signs and symptoms	ADAMTS13 activity by rF-vWF73, %	Plasma anti-ADAMTS13 inhibitors	Plasma anti-ADAMTS13IgG, U/mL	WT	M1	M2	M3	M4	M5
1	44	Female	15	23	695	1.1	Yes	< 5	+	84.2	++	++	++	+	−	−
2	56	Female	19	29	654	1.1	Yes	< 5	+	35.4	++	++	++	+	+	+
3	45	Female	88	28	995	0.7	Yes	< 5	+	81.9	++	++	++	−	−	−
4	52	Female	52	20	866	1.1	Yes	< 5	+	132.0	++	++	++	−	−	−
5	79	Female	7	27	1660	1.4	Yes	< 5	+	118.0	++	++	++	+	−	−
6	34	Male	15	23	2703	0.9	Yes	< 5	+	127.5	++	++	++	+	−	−
7	34	Male	0	33	859	1.9	No	< 5	+	162.0	++	++	++	−	−	−
8	23	Female	11	18	3412	0.8	Yes	< 5	+	132.0	++	++	++	−	−	−
9	42	Female	23	17	3259	0.8	Yes	< 5	+	168.0	++	++	++	−	−	−
10	21	Female	9	13	1489	1.5	Yes	< 5	+	72.0	++	++	++	+	+	+
11	61	Female	33	28	511	0.8	Yes	< 5	+	221.0	++	++	++	−	−	−
12	42	Male	16	24	6517	1.2	No	< 5	+	147.2	++	++	++	+	−	−
N = 12	43*		15.5*	23.5*	1242*	1.1*	(83.3)	(100)	(100)	(100)	(100)	(100)	(100)	(50)	(17)	(17)

Values in parentheses indicate positive rates in percentages.

LDH indicates lactate dehydrogenase; −, negative inhibition (< 10% reduction in activity); +, mild inhibition (10%-30% reduction in activity); ++, moderate inhibition (30%-50% reduction in activity); and +++, strong inhibition (> 50% reduction in activity) after 50:50 mixing with patient plasma.

Median values.

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