. Author manuscript; available in PMC: 2017 Oct 1.

Published in final edited form as: J Thromb Haemost. 2016 Sep 17;14(10):1931–1940. doi: 10.1111/jth.13438

Table 3.

Raw data for patients who tested positive for one or more anti-factor IX immunoglobulins by fluorescence immunoassay. Positive results in bold.

Patient	Severity	History of inhibitor	F9 mutation (type)	Exposure days^†	Interval days since first draw	IgG₁	IgG₂	IgG₃	IgG₄	IgA	IgE	NBU
A. Patients with positive Nijmegen-Bethesda assay

1	severe	ND	c.880C>T (nonsense)	< 21	0	9	13.5	9.5	12	100.5	11.5	0.3^*

2	ND	yes	c.223C>T (nonsense)	< 21	0	286.8	44.3	169.8	6420.8	527.8	66.5	0.3^*

3	severe	No	c.1150C>T (nonsense)	>150	0	10.3	14.5	8.8	15.5	472	13.5	0.1
					384	12	15.5	9.3	13.5	491.3	9	0.1
					762	9.5	12.8	6.5	13.5	308.3	16	0.0
					1126	10	12.8	7.5	12.3	331.5	16	0.1
					1504	9.8	9.5	7.3	11.3	89	11	0.4^*
					1875	10.5	14	9.3	12.5	261.3	15	0.1

4	severe	Yes	Deletion of exon 1–8	< 21	0	281.5	19	10	115.3	375.5	23.5	0.6^*
					281	2850	462.3	594.3	13631.3	2089.8	30	21.3
					399	1455	667.3	58	1324	1791.8	33.5	1.6

5	severe	Yes	Deletion of exon 1–5	101–150	0	1582	53.5	25.5	27231	4043	31.8	3.1^*
5	severe	Yes	Deletion of exon 1–5	101–150	462	195.3	1838.5	23.3	28648.8	9379.5	29.5	23.3

6	severe	Yes	Deletion of exon 1–8	>150	0	1142.5	145.3	13.5	25243.8	5862.3	37	0.8^*

7	severe	Yes	Deletion of exon 1–8	< 21	0	858.3	191.3	9.5	24839.3	1182.5	64	1.3^*

8	severe	Yes	c.659C>G (nonsense)	< 21	0	2512.3	501.5	5219	11112	2674.3	27	1.9^*

9	severe	Yes	Deletion of exon 1–8	21–100	0	165.3	51.3	15.8	26332	1423	86.5	3.2^*

10	ND	Yes	c.223C>T (nonsense)	< 21	0	1431	160.5	170.5	4689.5	2364	31.5	9.1^*

11	severe	Yes	c.223C>T (nonsense)	21–100	0	300	773.8	16	26511	6386.3	73.8	12.0^*

12	severe	Yes	c.880C>T (nonsense)	ND	0	993.8	1056.3	604.3	27614.5	4610.8	27	57.2^*

B. Patients with negative Nijmegen-Bethesda assay

13	severe	No	c.1228G>T (missense)	>150	0	10	10	8	11	133	16.3	0.1^*

14	mild	No	c.1136G>A (missense)	>150	0	11.5	25.5	8.5	8	618.5	20.5	0.2^*

15	severe	No	c.677G>A (missense)	>150	0	9.5	18	9.5	8.5	51.3	13.3	0.0^*
					462	7.8	8.8	7.5	8	61.3	10.3	0.1
					1870	7.5	12.5	8.5	9	57.3	12.3	0.0

16	moderate	No	c.1328T>C (missense)	21–100	0	9	10.3	9.5	8.8	340.5	33	0.0^*
					323	8	11.3	9.5	9	357.5	28.5	0.0
					750	10.3	13	6.8	8.3	350.3	19.8	0.0
					1590	9.5	10.8	9.5	8.3	364.3	24	0.0

17	mild	No	c.316G>A (missense)	21–100	0	99.5	59.5	8	8.5	156.3	11.5	0.0^*
17	mild	No	c.316G>A (missense)	21–100	631	20	17.3	8.3	8.5	80	11	0.0

18	moderate	No	c.1025C>T (missense)	21–100	0	80	11.3	11	49	618.5	15	0.0^*
					365	77.8	15	8.5	98.3	296	12.3	0.0
					771	ND	ND	ND	ND	ND	ND	0.1
					1128	62.3	9.5	5.5	41.5	413.8	12.5	0.0
					1674	67.3	9.5	7.5	40.8	502.5	14.5	0.0

19	moderate	No	c.786T>G (missense)	21–100	0	13	10.5	9	8.3	431	12	0.0
					365	91.5	16.3	9.3	8	370	12.8	0.0^*
					857	11.8	14.3	8.5	9	462.5	14.3	0.0
					1256	10	11	10	9.3	205.8	11.5	0.0
					1625	10	18.3	11.5	7.8	310.3	13	0.0

20	mild	No	c.572G>A (missense)	< 21	0	10.3	11.3	8.5	9.8	555.3	51.3	0.1^*
					372	9.5	14	7.8	7.8	686.5	46.3	0.0
					736	8.3	13	8.5	8.5	417.8	32	0.0

21	moderate	No	c.-35G>A (5′ UTR)	< 21	0	12.3	17.8	8.3	9	185.5	14	0.1^*
21	moderate	No	c.-35G>A (5′ UTR)	< 21	386	12.8	20.8	7	7.3	162.8	9.8	0.1

22	moderate	No	c.1025C>T (missense)	< 21	0	8.5	9.5	8.5	10.8	137.8	9.8	0.1
					372	9	10	8.5	10.5	340	16.3	0.0
					736	8.3	9.5	7.3	9.3	252.3	17	0.0
					1100	9	8.5	8.3	10	767	31.8	0.2^*
					1828	9.5	12.3	8.5	10.5	262.3	17.3	0.0
					2192	9.5	11	8.5	11.3	248	14	0.0

					Threshold for positivity	13.6	15.0	15.4	9.8	600.5	24.6	0.3

ND - No data collected; UTR - untranslated region;

sample included in analyses shown in Figures 2 and 3 and Table 2;

^†

Exposure days at study enrollment.