Table 3.
Laboratory findings of RAG deficient patients
| Patients | WBC (/mm³) |
ALC (/mm³) |
ANC(/mm³) | AEC (/mm³) |
IgA (mg/dl) | IgG (mg/dl) | IgM (mg/dl) | IgE (IU/l) | CD3** | CD4** | CD8** | CD16 + 56** | CD19** |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| SCID | |||||||||||||
| P1 | 8900 | 4806 | 1900 | 800 | 251 7–123 |
4090 304–1231 |
1 32–203 |
<1 | 17/49–76 817 1900–5900 |
11/31–56 528 1400–4300 |
21/12–24 1009 500–1700 |
79/3–15 3796 160–950 |
1/14–37 48 610–2600 |
| P2 † | 4100 | 984 | 2700 | 80 | 27 13.5–72 |
88 294–1165 |
70 33–154 |
N/A | 1.8/51–77 18 2500–5600 |
1.9/35–56 19 1800–4000 |
41/12–23 403 590–1600 |
73.2/3–14 718 170–830 |
2.6/11–41 26 430–3000 |
| P3 † | 6300 | 1000 | 3300 | 1400 | <6.67 11–14 |
402 633–1466 |
<4.17 22–87 |
<1 | 2/53–84 20 2500–5500 |
N/A | N/A | N/A | 2/6–32 20 300–2000 |
| P4 | 5400 | 1200 | 2800 | 500 | 9,7 7–123 |
11 304–1231 |
358.2 32–203 |
8.8 | 28/49–76 336 1900–5900 |
16.7 200 1400–4300 |
27/12–24 324 500–1700 |
57.7/3–15 692 160–950 |
0.2/14–37 2.4 610–2600 |
| P5 | 3800 | 700 | 800 | 400 | 0 7–123 |
177 304–1231 |
2 32–203 |
<1 | 0/49–76 0 1900–5900 |
3/31–56 21 1400–4300 |
46/12–24 322 500–1700 |
79/3–15 553 160–950 |
0/14–37 0 610–2600 |
| P6 † | 4700 | 940 | 2500 | 0 | <6.67 7–123 |
404 304–1231 |
<4.17 32–203 |
<1 | 0/49–76 0 1900–5900 |
7/31–56 66 1400–4300 |
32/12–24 300 500–1700 |
66.8/3–15 628 160–950 |
0/14–37 0 610–2600 |
| P7 † | 1100 | 300 | 400 | 0 | 18 13.5–72 |
120 294–1165 |
11 33–154 |
<1 | 0/51–77 0 2500–5600 |
3/35–56 9 1800–4000 |
44/12–23 132 590–1600 |
95/3–14 285 170–830 |
0/11–41 0 430–3000 |
| P8 | 17000 | 1000 | 15600 | 300 | 8 13.5–72 |
580 294–1165 |
16 33–154 |
0/53–84 0 2500–5500 |
2/35–64 20 1600–4000 |
26/12–28 260 560–1700 |
57/4–18 570 170–1100 |
0/6–32 0 300–2000 |
|
| P9 | 2800 | 400 | 1500 | 100 | 21 7–123 |
110 304–1231 |
22 32–203 |
<1 | 2/49–76 8 1900–5900 |
5/31–56 20 1400–4300 |
46/12–24 184 500–1700 |
86/3–15 344 160–950 |
0/14–37 0 610–2600 |
| P10 | 3200 | 400 | 1700 | 100 | 21 26–296 |
510 604–1941 |
25 71–235 |
100 | 10/56–75 40 1400–3700 |
17/28–47 68 700–2200 |
33/16–30 132 490–1300 |
87/4–17 348 130–720 |
0/14–33 0 390–1400 |
| P11 | 20300 | 10200 | 8932 | 100 | 47.3 17–107 |
641 463–1006 |
39 46–159 |
11.3 | 11.3/49–76 1153 1900–5900 |
7.38/31–56 752 1400–4300 |
24.5/12–24 2499 500–1700 |
70.8/3–15 7221 160–950 |
3.69/14–37 376 610–2600 |
| P12 | 6300 | 1260 | 4400 | 500 | 35 13.5–72 |
120 294–1165 |
6 33–154 |
<1 | 0.9/51–77 11 2500–5600 |
1.8/35–56 24 1800–4000 |
41.5/12–23 529 590–1600 |
84.7/3–14 1071 170–830 |
0.7/11–41 10 430–3000 |
| P13 | 4100 | 100 | 3000 | 0 | 24 13.5–72 |
420 294–1165 |
17 33–154 |
18.3 | 0/51–77 0 2500–5600 |
0.4/35–56 0.4 1800–4000 |
0.4/12–23 0.4 590–1600 |
93/3–14 93 170–830 |
0.4/11–41 0.4 430–3000 |
| P14 | 100 | 0 | 0.1 | <22 7–123 |
884 304–1231 |
19 33–154 |
<18 | 13/49–76 0 1900–5900 |
0/31–56 0 1400–4300 |
39/12–24 0 500–1700 |
57/3–15 0 160–950 |
0/14–37 0 610–2600 |
|
| P15 | 2800 | 900 | 1400 | 0 | <6.67 13.5–72 |
69.9 294–1165 |
<4.17 33–154 |
<1 | 0/51–77 0 2500–5600 |
1/35–56 9 1800–4000 |
16/12–23 144 590–1600 |
86/3–14 774 170–830 |
0/11–41 0 430–3000 |
| P16 * | 4600 | 800 | 2900 | 100 | <6.67 13.5–72 |
136 294–1165 |
<4.17 33–154 |
0.1/51–77 0.8 2500–5600 |
51.1/35–56 1800–4000 |
25,2/12–23 201 590–1600 |
57,1/3–14 456 170–830 |
0.64/11–41 5 430–3000 |
|
| P17 * | 1700 | 200 | 1200 | 0 | <6.67 13.5–72 |
755 294–1165 |
<4.17 33–154 |
<1 | 1/51–77 2 2500–5600 |
0/35–56 0 1800–4000 |
3/12–23 6 590–1600 |
9/3–14 18 170–830 |
4/11–41 8 430–3000 |
| P18 | 11900 | 900 | 9300 | 700 | 225 17–69 |
1150 463–1006 |
168 46–159 |
935 | 16/49–76 144 1900–5900 |
13/31–56 117 1400–4300 |
27/12–24 243 500–1700 |
67/3–15 603 160–950 |
8/14–37 72 610–2600 |
| P19 | 3400 | 400 | 2000 | 200 | 18 13.5–72 |
290 294–1165 |
8 33–154 |
<1 | 16/53–84 64 2500–5500 |
19/35–64 76 1600–4000 |
20/12–28 80 560–1700 |
64/4–18 256 170–1100 |
0.6/6–32 2.4 300–2000 |
| P20 | 7700 | 1100 | 5400 | 0 | <6.67 13.5–72 |
137 294–1165 |
<4.17 33–154 |
66 | 2/51–77 22 2500–5600 |
1/35–56 11 1800–4000 |
24/12–23 264 590–1600 |
65/3–14 715 170–830 |
2/11–41 22 430–300 |
| P21 | 5100 | 900 | 2900 | 200 | <6.67 13.5–72 |
102 294–1165 |
6.33 33–154 |
<1 | 0/51–77 0 2500–5600 |
73/35–56 63 1800–4000 |
13/12–23 117 590–1600 |
72/3–14 648 170–830 |
1/11–41 9 430–300 |
| P22 | 7400 | 1100 | 4000 | 0 | <6.67 13.5–72 |
191 294–1165 |
<4.17 33–154 |
<1 | 4/53–84 44 2500–5600 |
6/35–64 66 1800–4000 |
8/12–28 88 590–1600 |
66/4–18 726 170–830 |
4/6–32 44 430–300 |
| P23 | 4600 | 800 | 3600 | 100 | <6.67 13.5–72 |
262 294–1165 |
<4.17 33–154 |
<1 | 1/53–84 8 2500–5600 |
3/35–64 24 1800–4000 |
26/12–28 208 590–1600 |
85/4–18 680 170–830 |
0/6–32 0 430–300 |
| P24 | 8400 | 1300 | 4500 | 1200 | <6.67 9–30 |
367 376–685 |
44,1 36–77 |
43,1 | 36/53–84 468 2500–5500 |
36/35–64 468 1600–4000 |
26/12–28 338 560–1700 |
56/4–18 728 170–1100 |
0/6–32 0 300–2000 |
| P25 | 11600 | 100 | 9600 | 500 | <6.67 11–14 |
888 633–1466 |
<4.17 22–87 |
<1 | 64/53–84 64 2500–5500 |
10/35–64 10 1600–4000 |
54/12–28 54 560–1700 |
28/4–18 28 170–1100 |
0/6–32 0 300–2000 |
| OS | |||||||||||||
| P26 † | 25600 | 10240 | 3072 | 2560 | 17.7 13.5–72 |
126 294–1165 |
10.7 33–154 |
<1 | 9/49–76 921 2500–5600 |
9/31–56 921 1800–4000 |
23/12–24 2355 590–1600 |
83/3–15 8397 170–830 |
0/14–37 0 430–3000 |
| P27 † | 12200 | 1220 | 7320 | 1220 | <6.67 13.5–72 |
226 294–1165 |
<4.17 33–154 |
<1 | 21/53–84 240 2500–5500 |
17/35–64 200 1600–4000 |
28/12–28 380 560–1700 |
68/4–18 850 170–1100 |
0/6–32 0 300–3000 |
| P28 | 7200 | 2400 | 1400 | 500 | 11 17–69 |
61 463–1006 |
32 32–203 |
>1000 | 84.2/49–76 2016 1900–5900 |
41.1/31–56 984 1400–4300 |
52.7/12–24 1264 500–1700 |
13.5/3–15 324 160–950 |
0.5/14–37 12 610–2600 |
| P29 | 20500 | 2240 | 15000 | 470 | <30 17–69 |
<160 463–1006 |
<22 32–203 |
5 | 31/49–76 694 1900–5900 |
29/31–56 650 1400–4300 |
24/12–24 537 500–1700 |
53/3–15 1187 160–950 |
0/14–37 0 610–2600 |
| P30 | 4900 | 500 | 1300 | 2600 | <6.67 13.5–72 |
1180*** 294–1165 |
5.84 33–154 |
<1 | 16/51–77 80 2500–5500 |
17/35–64 200 1600–4000 |
24/12–23 120 560–1700 |
64/3–14 320 170–1100 |
2/11–41 10 300–3000 |
| CID | |||||||||||||
| P31* | 19700 | 1000 | 17500 | 100 | 208 70–303 |
1040 764–2134 |
150 69–387 |
5.37 | 45/60–76 450 1200–2600 |
19/31–47 190 650–1500 |
24/18–35 240 370–1100 |
46/4–17 460 100–480 |
8/13–27 80 270–860 |
| P32* | 5300 | 1000 | 3500 | 200 | 48,7 57–282 |
939 745–1804 |
154 78–261 |
14.4 | 21/56–75 210 1400–3700 |
13/28–47 130 700–2200 |
28/16–30 280 490–1300 |
40/4–17 400 130–720 |
8/14–33 80 390–1400 |
| P33 | 4400 | 2600 | 1000 | 0 | 12 17–69 |
560 463–1006 |
11 46–59 |
<1 | 49/49–76 1274 1900–5900 |
36/31–56 936 1400–4300 |
9/12–24 234 500–1700 |
44/3–15 1144 160–950 |
1/14–37 26 610–2600 |
| P34 | 10500 | 1000 | 9100 | 0 | 36.2 26–296 |
1020 604–1941 |
47.3 71–235 |
255 | 45/56–75 450 1400–3700 |
23/28–47 230 700–2200 |
35/16–30 350 490–1300 |
36/4–17 360 130–720 |
9/14–33 90 390–1400 |
| P35 | 2200 | 300 | 900 | 0 | 11 30–107 |
14 605–1430 |
8 66–228 |
<1 | 40/53–75 120 2100–6200 |
21/32–51 63 1300–3400 |
32/14–30 96 620–2000 |
38/3–15 114 180–920 |
0/16–35 0 720–2600 |
† : cousins: P2 and P3; P6 and P7; P26 and P27; *: siblings: P16 and P17; P31 and P32; **: [%-/mm3)]; ***: after IVIG treatment; N/A: not applicable.
AEC: Absolute eosinophil count; ALC: Absolute lymphocyte count; ANC: Absolute neutrophil count; WBC: White blood cell.
All of the labs were measured in the first visit.