Baerbel Keller, Irina Zaidman, O. Sascha Yousefi, Dov Hershkovitz, Jerry Stein, Susanne Unger, Kristina Schachtrup, Mikael Sigvardsson, Amir A. Kuperman, Avraham Shaag, Wolfgang W. Schamel, Orly Elpeleg, Klaus Warnatz, Polina Stepensky

Online ISSN: 1540-9538

Print ISSN: 0022-1007

2017

J Exp Med (2017) 214 (7): 2165.

https://doi.org/10.1084/jem.2015111005242017c

Vol. 213, No. 7, June 27, 2016. https://doi.org/10.1084/jem.20151110

The authors regret that in the original version of their paper, two values for patient 2 in Table 2 were incorrect. The corrected values are red in the table below.

This has been corrected in the online HTML and PDF versions. The error remains only in the print version.

View Original Article

2017

Table 2.

Immune phenotype of patient 1, 2, and 3

	Patient 1 (6 yr)				Patient 2 (8 yr)				Patient 3 (2 yr)
	Relative counts	Reference values	Absolute	Reference values	Relative counts	Reference values	Absolute	Reference values	Relative counts	Reference values	Absolute	Reference values
Lymphocytes ^{^a}			1,855	1,800–5,000			900	1,800–5,000			4,950	2,800–6,400
B cells ^{^a}	2.0	8.5–20.2	37	296–784	3.8	8.5–20.2	34	296–784	29.9	17.3–30.0	1,480	686–1,732
CD4 ^{^a}	4.0	26.5–41.4	74	641–1,453	24.5	26.5–41.4	220	641–1,453	6.4	28.1–43.2	317	925–2,477
CD8 ^{^b}	45.0	13–47	834	200–1,700	38.3	13–47	345	200–1,700	4.5	9–49	223	200–1,800
NK cells ^{^b}	6.0	2–31	114	70–590	1.7	2–31	16	70–590	0.9	2–25	45	61–510
B cell subpopulations ^{^a}	ND		ND						ND		ND
Transitional					21.1	3.4–9.0	5	13–63
Naive					68.7	47.8–69.8	23	154–413
IgM memory					3.5	6.3–22	2	24–135
IgM only					0.7	2–11.8	1	7–65
IgA cs memory					0	1.1–6.1	0	5–35
IgG cs memory					0	2.7–14	0	13–74
CD21^low					6.3
CD4 subpopulations ^{^a}	ND		ND						ND		ND
RTE^{^c}					24.4	61–84.2	1
Naive					2.4	55.6–75.8	5	375–1,096
CD45R0					97.5	24–43
T reg cells					2.0	2.3–7.7	5	18–86
cTFH^{^d}					35.0	18.4–29.9	112	51–218
Th₂⁺Th₁₇-like cTFH^{^d}					29.4	39.9–66.1	33	26–85
CD8 subpopulations ^{^b}	ND		ND						ND		ND
Naive					0.3	16–100	1	42–1,300
Central memory					2.4	1–6	8	6–43
Effector memory					65.9	5–100	227	45–410
Terminally differentiated					1.6	15–41	6	57–340
γδ T cells ^{^e}	ND		ND		37.2	<10	282	12–175	72	<10	2,217	12–175
DN T cells ^{^e}	51				38				72
NK T cells ^{^e}	ND		ND		0.005	>0.01	ND		ND		ND

	Patient 1 (6 yr)				Patient 2 (8 yr)				Patient 3 (2 yr)
	Relative counts	Reference values	Absolute	Reference values	Relative counts	Reference values	Absolute	Reference values	Relative counts	Reference values	Absolute	Reference values
Lymphocytes ^{^a}			1,855	1,800–5,000			900	1,800–5,000			4,950	2,800–6,400
B cells ^{^a}	2.0	8.5–20.2	37	296–784	3.8	8.5–20.2	34	296–784	29.9	17.3–30.0	1,480	686–1,732
CD4 ^{^a}	4.0	26.5–41.4	74	641–1,453	24.5	26.5–41.4	220	641–1,453	6.4	28.1–43.2	317	925–2,477
CD8 ^{^b}	45.0	13–47	834	200–1,700	38.3	13–47	345	200–1,700	4.5	9–49	223	200–1,800
NK cells ^{^b}	6.0	2–31	114	70–590	1.7	2–31	16	70–590	0.9	2–25	45	61–510
B cell subpopulations ^{^a}	ND		ND						ND		ND
Transitional					21.1	3.4–9.0	5	13–63
Naive					68.7	47.8–69.8	23	154–413
IgM memory					3.5	6.3–22	2	24–135
IgM only					0.7	2–11.8	1	7–65
IgA cs memory					0	1.1–6.1	0	5–35
IgG cs memory					0	2.7–14	0	13–74
CD21^low					6.3
CD4 subpopulations ^{^a}	ND		ND						ND		ND
RTE^{^c}					24.4	61–84.2	1
Naive					2.4	55.6–75.8	5	375–1,096
CD45R0					97.5	24–43
T reg cells					2.0	2.3–7.7	5	18–86
cTFH^{^d}					35.0	18.4–29.9	112	51–218
Th₂⁺Th₁₇-like cTFH^{^d}					29.4	39.9–66.1	33	26–85
CD8 subpopulations ^{^b}	ND		ND						ND		ND
Naive					0.3	16–100	1	42–1,300
Central memory					2.4	1–6	8	6–43
Effector memory					65.9	5–100	227	45–410
Terminally differentiated					1.6	15–41	6	57–340
γδ T cells ^{^e}	ND		ND		37.2	<10	282	12–175	72	<10	2,217	12–175
DN T cells ^{^e}	51				38				72
NK T cells ^{^e}	ND		ND		0.005	>0.01	ND		ND		ND

Relative counts are shown as a percentage of parental population. Absolute numbers refer to cells/microliter. CD4 T cell subpopulations: recent thymic emigrants (RTEs), CD31⁺ of CD45RA⁺ CD4 T cells; T reg cells, CD127⁻ CD25hi of CD45RA⁻ CD4 T cells; circulating T follicular helper–like cell (cTFH), CXCR5⁺ of CD45RA⁻ CD4 T cells; Th2/Th17-like cTFH, CXCR3⁻ of CXCR5⁺ CD45RA⁻ CD4 T cells. CD8 T cell populations: naive, CCR7⁺ CD45RA⁺ CD27⁺; terminally differentiated, CCR7⁻ CD45RA⁺ CD27⁻; central memory, CCR7⁺ CD45RA⁻ CD27⁺; effector memory, CCR7⁻ CD45RA⁻ CD27⁻. Double-negative (DN) T cells, CD4⁻/CD8⁻ of CD3 T cells. NK T cells, Vα24 Vβ11 CD3 T cells. cs, class switched.

van Gent et al., 2009.

Schatorjé et al., 2012.

Percentage of naive CD4 T cells.

Internal reference value.

Internal reference value of CD3 T cells.

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Correction: Early onset combined immunodeficiency and autoimmunity in patients with loss-of-function mutation in LAT

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