. Author manuscript; available in PMC: 2021 Jan 1.

Published in final edited form as: J Allergy Clin Immunol. 2019 Oct 7;145(1):358–367.e2. doi: 10.1016/j.jaci.2019.09.020

Table II.

Patient Immunophenotyping

Subject Number:	A.I.1	A.II.1	A.II.2	A.II.3	A.II.4	A.II.5	B.II.2	C.II.2	D.I.2	D.II.3	E.I.1	E.II.2
TRECs/mm³	ND	ND	ND	< 1	5.2	ND	< 5	14.2	ND	9.7	ND	ND

Initial Flow Cytometry (age)	31 yr	6 yr 10 mo	45 mo	3 mo	3 mo	3 mo	1 mo	2 wk	23 yr	2 wk	28 yr	4.5 mo
% CD3	59	58^*	51^*	6^*	21^*	36^*	3^*	31^*	78	49^*	79	35^*
% CD4	32	32	34	5^*	18^*	24^*	3^*	22^*	30	28^*	31	21^*
%CD8	25	18	11^*	0.3^*	2^*	9^*	0.1^*	9^*	33	19	37	11^*
CD4/CD8 (1.5-2.5)	1.28	1.75	3.12^**	15.37^**	7.48^**	2.5	34.52^**	2.37	0.93^*	1.48	0.85	1.89
Abs CD3/mm³	1300	1413	1164^*	308^*	916^*	1928^*	91^*	1655^*	1423	1377^*	1132	1307^*
Abs CD4/mm³	700	774	772	273^*	796	1299^*	91^*	1158^*	544	734^*	444	794^*
Abs CD8/mm³	547	435	250^*	16^*	88	487^*	3^*	474^*	599	480^*	530	416^*

% CD19	13	22	20	78^**	56^**	51^**	51^*	8^*	10	34^**	13	54^**
Abs CD19/mm³	275	534	455	4272^**	2498^**	2775^**	1431	405	191	971	182	2039
kappa/lambda (1.4-1.9)^‡	0.66^*	0.68^*	0.74^*	0.9^*	0.99^*	1.08^*	0.47^*	0.42^*	1.15^*	0.54^*	1.84	1.4

% CD3−/CD16+/CD56+	24	17	25^**	15	19^**	9	46^**	58^**	4.6	11	8	9
Abs CD3−/CD16+/CD56+	525	411	568^**	820	840	487	1291^**	3055^**	83	311	115	340

Immunoglobulins				2 yr	2 yr	2 yr	2 yr	7 mo
IgG	ND	ND	ND	730^***	463	551	453	431	ND	ND	ND	662
IgA	ND	ND	ND	33	17	35	40	33	ND	ND	ND	36
IgM	ND	ND	ND	83	73	64	28^*	54	ND	ND	ND	93
IgE	ND	ND	ND	23	3	5	57	ND	ND	ND	ND	ND

Vaccine responses	ND	ND	ND	28 mo	7 mo	7 mo	26 mo	7 mo	ND	ND	ND	11 mo
Tetanus^#				ND	3.89	3.26	0.52	1.12				0.2
Pneumococcal^§ (Number protective of 14)				13 of 14	10 of 14	10 of 14	8 of 14	8 of 14				10 of 14

Low for age (Reference: Shearer et al 2003. Lymphocyte subsets in healthy children to 18 years)

^**

High for age

^***

on IgG replacement

^‡

Deneys et al, 2001. J Immunol Meth 253:23-36.

Protective ≥ 0.15 μg/Ml

^§

Protective ≥ 1.3 μg//mL

ND: Not Done