. Author manuscript; available in PMC: 2015 May 1.

Published in final edited form as: J Allergy Clin Immunol. 2014 Feb 28;133(5):1400–1409.e5. doi: 10.1016/j.jaci.2014.02.013

Table II.

Significant immune laboratory findings of individuals with hypomorphic PGM3 mutations.

		Immunoglobulins				CBC				CD3			CD20		NK	EBV	RF
	Patient	IgE IU/mL (0–90)	IgG mg/dL (759–1,550)	IgA mg/dL (58–359)	IgM mg/dL (35–239)	WBC 10³ cells/ µL	ANC 10³cells/ µL	ALC 10³cells/ µL	AEC 10³cells/ µL	CD4 cells/µL (359–1,565)	CD8 cells/µL (178–853)	CD4/CD 8 ratio	Total cells/µL (61–321)	CD27⁺ Memory cells/µL (12–68)	cells/µL (126–729)	10³ copies/ mL	(<15 IU/mL)
Family I	I.1	11,200	1610–1,770	284–547	41–74	3.1–5.3	2.1–3.3	0.6–0.9	0.1–0.5	128	129	1	253	7	113	5.0	POS
	I.2	5,836–27,200	1,510–1,890	749–1,660	125–176	4.8–9.2	2.9–7.1	0.6–1.1	0.2–0.5	195–487	70–137	2.8–3.6	31–161	7	202–270	30.0	POS
	I.3	4,672–35,400	422–1,750	515–1,140	121–411	1.7–5.5	0.9–4.4	0.2–0.8	0.0–1.1	93–151	11–19	8–11	100–123^**	2–3^**	15–62	2.2	POS
	I.4	23,594–35,445	673–1,470	509–877	103–130	2.7–4.7	1.4–3.5	0.3–0.7	0.0–0.9	150–248	22–43	5.8–6.8	37–140^**	4–10^**	30–131	28.2	POS
	I.5	ND	ND	ND	ND	11.4–19.3	11–12.0	0.6–11.4	0–3.6	ND	ND	ND	ND	ND	ND	ND	ND
Family II	II.1	1,244–30,766	1,080–1,690	334–497	86–147	1.3–5.4	0.1–3.3^*	0.7–1.3	0–0.4	368–692 ^§	111–247^§	2.8–5.1^§	16–108^§	1^§	150–260^§	NEG	NEG
	II.2	5,396–11,405	1,660–2,180	407–677	42–144	1.0–3.5	0.4–1.7	0.4–2.0	0–0.2	403–878	189–295	2.1–3.0	4–112	1	160–503	naive	POS
	II.3	207	554	84	38	1.9–5.5	0.7–1.0	0.4–2.3	0–1.7	324 (1,300–4,300) ^†	263 (500–2,000) ^†	1.23	711 (773–1,990) ^†	11 (56–172) ^†	664 (160–920) ^†	ND	ND ^*

All values within normal range were obtained while receiving G-CSF;

^§

flow data obtained during acute meningoencephalitis were excluded;

^**

received rituximab historically, prior to complex immunophenotyping; rheumatoid factor (RF) is reported as positive if ever detected in peripheral blood;

^†

normal pediatric values for 1 year old 19, 20