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. 2021 Jul 6;41(7):1515–1522. doi: 10.1007/s10875-021-01065-9

Table 2.

Immunological evaluation of the patients with combined immunodeficiencies

Patients Absolute lymphocyte count (ALC) (/mm3) Immunoglobulins (mg/dL) Lymphocyte subsets %/absolute counts (/mm3) T and B cell subpopulations Lymphocyte activation/transformation
P1 (RASGRP1 deficiency) ALC: 4064 (1100–5900)

IgA: 660 (70–303)

IgG:2030 (764–2134)

IgM:188 (69–387)

Total IgE: < 1.00 IU/mL

CD3: 64 (55–78)

2600 (700–4200)

CD4: 13 (27–53)

528 (300–2000)

CD8:55 (19–34)

2235 (300–1800)

CD16 + 56: 22(4–26)

894 (90–900)

CD19:10(10–31)

406 (200–1600)

 ND Low
P2 ALC: 1000 (1900–3700)

IgA: 134 (62–390)

IgG:984 (842–1943)

IgM:62.9 (54–392)

Total IgE: 13.7 IU/mL

CD3: 68 (60–76)

680 (1200–2600)

CD4: 20(31–47)

200 (650–1500)

CD8:50 (18–35)

500 (370–1100)

CD16 + 56: 15 (4–17)

150 (100–480)

CD19:8 (13–27)

80 (270–860)

Low memory and switch memory B cells

Low naive T cells, increased effector memory T cells

RTE: 6.5% (7–100)

 Low
P3 ALC: 1700 (3600–8900)

IgA: 9.53 (30–107)

IgG:2140* (605–1430)

IgM:10.5 (66–228)

Total IgE: < 1.00 IU/mL

CD3: 87 (53–75)

1479 (2100–6200)

CD4: 57 (32–51)

969 (1300–3400)

CD8:35 (14–30)

595 (620–2000)

CD16 + 56: 9(3–15)

153 (180–920)

CD19:1(16–35)

17 (720–2600)

 ND

CD3: 17 (59–80.7)

CD25: 17 (86–99.8)

CD69: 25 (61.2–91.8)

CD3 + CD25 + : 13 (52–93)

CD3 + CD69 + :14 (57–84)
P4 (STAT1 GOF) ALC: 1100 (1900–3700)

IgA: 142 (70–303)

IgG:940 (764–2134)

IgM:64.9 (69–387)

Total IgE: 1.23 IU/mL

CD3: 59 (60–76)

649 (1200–2600)

CD4: 28 (31–47)

308 (650–1500)

CD8:27 (18–35)

297 (370–1100)

CD16 + 56: 19 (4–17)

209 (100–480)

CD19:15 (13–27)

165 (270–860)

Low memory and switch memory B cells

Low naive T cells, increased effector memory T cells

RTE: 28 (41–81)

CD3: 35 (45–74)

CD25: 58 (67–98)

CD69: 17 (70–83)

CD3 + CD25 + :18(46–88.5)

CD3 + CD69 + :15(50–75.6)
P5 (EBV + lymphoma) ALC: 2100 (1400–3300)

IgA: 120 (139–378)

IgG:410 (913–1884)

IgM:17.3 (88–322)

Total IgE: 1.43 IU/mL

CD3: 95 (56–84)

1995 (1000–2200)

CD4: 81 (31–52)

1701 (530–1300)

CD8:14 (18–35)

294 (330–920)

CD16 + 56: 1 (3–22)

21 (70–480)

CD19:3 (6–23)

63 (110–570)

Low naive T cells, increased effector memory T cells

RTE: 1 (7–100)

CD3: 44 (45–74)

CD25: 38 (67–98)

CD69: 25 (70–83)

CD3 + CD25 + : 35 (46–88.5)

CD3 + CD69 + : 15 (50–75.6)
P6 (CARD11 deficiency) ALC: 2500 (1400–3300)

IgA: 214 (139–378)

IgG:1090 (913–1884)

IgM:231 (88–322)

Total IgE: 154 IU/mL

CD3: 74 (56–84)

1850 (1000–2200)

CD4: 45 (31–52)

1125 (530–1300)

CD8:26 (18–35)

650 (330–920)

CD16 + 56: 8 (3–22)

200 (70–480)

CD19:14 (6–23)

375 (110–570)

Low naive T cells, increased effector memory T cells

RTE: 51 (7–100)

CD3: 49 (45–74)

CD25: 70 (67–98)

CD69: 82 (70–83)

CD3 + CD25 + : 45 (46–88.5)

CD3 + CD69 + : 45 (50–75.6)
P7 (AT) ALC: 2000 (1900–3700)

IgA: 8 (30–107)

IgG:280 (605–1430)

IgM:45 (66–228)

Total IgE: 1.61 IU/mL

CD3: 70 (56–84)

1400 (1000–2200)

CD4: 35 (31–52)

700 (530–1300)

CD8:42 (18–35)

840 (330–920)

CD16 + 56: 23 (3–22)

460 (70–480)

CD19:4 (6–23)

80 (110–570)

 ND ND
P8 (AT) ALC: 1400 (1900–3700)

IgA: 13.6 (30–107)

IgG:423 (605–1430)

IgM:154 (66–228)

Total IgE: 5.54 IU/mL

CD3: 68 (60–76)

952 (1200–2600)

CD4: 48 (31–47)

672 (650–1500)

CD8:15 (18–35)

210 (370–1100)

CD16 + 56: 17 (4–17)

238 (100–480)

CD19:13 (13–27)

182 (270–860)

 ND ND

The abnormal values are shown in bold