. 2020 Oct 17;9(10):3335. doi: 10.3390/jcm9103335

Table 4.

Immunological data of eight Italian APDS-1 patients at first evaluation.

		P1	P2	P3	P4	P5	P6	P7	P8
WBC ×10³/µL (4.0–10.8)		11.25	6.90	7.18	4.18	6.14	8.8	8.51	8.06
Neutrophils ×10³/µL (1.5–8.0)		9.40	3.40	4.93	2.93	4.58	5.33	6.43	4.90
Lymphocytes ×10³/µL (0.9–4.0)		1.51	2.30	1.42	0.785	1.21	2.38	1.27	2.12
IgG, mg/dL (700–1600)		855	1452	282 ^†	495	2119 ^ß‡	362 ^ƒ§	2056 ^ß‡	725 ^∂#
IgA, mg/dL (70–400)		227	80	55 ^†	10	207 ^ß‡	38 ^ƒ§	287 ^ß‡	15 ^∂#
IgM, mg/dL (40–230)		2332	145	170 ^†	7	127 ^ß‡	414 ^ƒ§	155 ^ß‡	228 ^∂#
Anti-HBs IgG (>10 IU/L)		30 IU/L	n.a.	absent	absent	584 IU/L	absent	n.a.	n.a.
Anti-tetanus IgG (>0.1 IU/mL)		0.5 IU/L	2.5 IU/L	absent	absent	0.6 IU/mL	absent	n.a.	1.0 UI/L
CFSE-base T cells Proliferation assay
	Anti-CD3	normal	n.a.	normal	n.a.	n.a.	CD4⁺ normalCD8⁺ reduced	n.a.	n.a.
	Anti-CD3 + IL-2	normal	n.a.	normal	n.a.	n.a.	normal	n.a.	n.a.
	PHA	reduced	n.a.	normal	n.a.	n.a.	CD4⁺ normalCD8⁺ reduced	n.a.	n.a.

Abbreviations: Anti-HBs IgG: Anti-Hepatitis B surface Immunoglobulin G, APDS-1: Activated Phosphoinositide 3-Kinase Delta Syndrome-1, CFSE: Carboxyfluorescein succinimidyl ester, IL-2: Interleukin-2, IgA: immunoglobulin A, IgG: immunoglobulin G, IgM: immunoglobulin M, PHA: Phytohemagglutinin, WBC: White Blood Cells. Notes: n.a.: not available; †: age adjusted reference value: IgG 528–1959 mg/dL, IgA 37–257 mg/dL, IgM 49–292 mg/dL; ß‡: age adjusted reference value: IgG 640–1909 mg/dL IgA 61–301 mg/dL, IgM 59–297 mg/dL; ƒ§: age adjusted reference value: IgG 633–2016 mg/dL, IgA 41–315 mg/dL, IgM 56–261 mg/dL; ∂#: age adjusted reference value: IgG 462–1710 mg/dL, IgA 27–173 mg/dL, IgM 62–257 mg/dL.