Table I.

Immunologic features of patient with NFκB2 LOF

Patient age (y)	Data	Patient result	Reference interval or control result
8 (at diagnosis)	IgG level (mg/dL) (L)	208 mg/dL	537-1,432
	IgA level (mg/dL) (L)	10 mg/dL	54-219
	IgM level (mg/dL) (L)	24 mg/dL	26-112
	Proportion of switched memory B cells (%)	0.2	Median: 20
	Tetanus titers before/after DTaP booster (IU/mL)	<0.1/0.7	≥0.1
	Diphtheria titers before/after DTaP booster (IU/mL)	<0.1/<0.1	≥0.1
	Baseline pneumococcal antibody levels (μg/mL)	For 15 of 23 serotypes, ≥1.3	For each serotype, ≥1.3
	Pneumococcal 12 serotype avidity panel (AI units)	for 6 of 12, <0.3; for all <1.0	0.3-4.0 (reportable range)
	CD45+ ALC (cells/μL)	2,091	1,500-6,800
	CD3+ T cells (cells/μL)	1,514	1,200-2,600
	CD4+ T cells (cells/μL)	1,222	650-1,500
	CD8+ T cells (cells/μL) (L)	259	370-1,100
	CD19+ B cells (cells/μL)	349	270-860
	CD4/CD8 ratio	4.7	>0.9
8 (at diagnosis)	Lymphocyte prolifer ation to mitogens∗	Unstimulated: 121 PHA: 291,693 ConA: 350,462 PWM: 156,686	0-286 cpm ≥135,190 cpm ≥73,522 cpm ≥26,677 cpm
	Lymphocyte proliferation to antigens∗	Unstimulated: 111 Candida: 2,606 (low) Tetanus: 207 (low)	≥15,289 cpm ≥4,761 cpm
17 (after COVID-19 infection; HD 42 from onset of illness)	Lymphocyte proliferation to mitogens†	% CD3+ T cells to PHA: 84.2% % CD3+ T cells to PWM: 18.6%	≥58.5% ≥3.5%
	Lymphocyte proliferation to anti-CD3 panel†	% CD3+ T cell to soluble anti-CD3: 45.9% % CD3+ T cell to soluble anti-CD3+ anti-CD28: 66.9% % CD3+ T cell to soluble anti-CD3+ IL-2: 59.7%	≥20.3% ≥44.6% ≥46.2%

AI, Avidity index; ALC, absolute lymphocyte count; ConA, concanavalin A; cpm, counts per minute; DTaP, diphtheria and tetanus toxoids and acellular pertussis; HD, healthy donor; L, low; PWM, pokeweek mitogen.

^∗Expressed as cpm of tritiated thymidine.

^†Expressed as percentage of proliferating CD3⁺ T cells relative to total CD3⁺ T cells.