| |
Case 1 |
Case 2 |
Case 3 |
Case 4 |
| Age at evaluation |
14 y |
8 y |
7 y |
8y |
| Lymphocyte count/mm3 × 10-3 |
7.44 (1.40-3.30) |
0.65 (1.90-3.70) |
1.4 (1.90-3.70) |
2.2 (1.90-3.70) |
| CD3pos cells/mm3 × 10-3 |
4.61 (1.00-2.20) |
0.24 (1.20-2.6) |
0.55 (1.20-2.6) |
1.8 (1.20-2.6) |
| CD4pos cells/mm3 × 10-3 |
2.3 (0.53-1.30) |
0.07 (0.65-1.50) |
0.29 (0.65-1.50) |
0.91 (0.65-1.50) |
| CD8pos cells/mm3 × 10-3 |
2.2 (0.33-0.92) |
0.13 (0.37-1.10) |
0.16 (0.37-1.10) |
0.53 (0.37-1.10) |
| CD19pos cells/mm3 × 10-3 |
2.7 (0.11-0.57) |
0.07 (0.27-0.86) |
0.18 (0.27-0.86) |
0.31 (0.27-0.86) |
| NK cells/mm3 × 10-3 |
0.22 (0.07-0.48) |
0.29 (0.1-0.48) |
0.30 (0.1-0.48) |
0.07 (0.1-0.48) |
| CD4/CD8 ratio |
1.0 |
0.54 |
1.5 |
1.7 |
Recent thymic emigrant cells mm3 × 10-3
CD31posCD45RAposCD4pos cells |
ND |
0.02 (0.05-0.93) |
ND |
ND |
Naive CD4 cells
CD45RAposCD45ROneg/CD4pos (%) |
39 (44-73) |
8 (44-73) |
47 (44-73) |
64 (44-73) |
Memory CD4 T cells
CD45ROposCD45RAneg/CD4pos (%) |
52 (12-42) |
87 (12-42) |
44 (12-42) |
24 (12-42) |
Naive CD8 cells
CCR7posCD45RAposCD27pos/CD8pos (%) |
21 (20-95) |
1 (16-100) |
36 (16-100) |
63 (16-100) |
Memory CD8 T cells
CCR7negCD45RAneg/CD8pos (%) |
11 |
50 |
28 |
22 |
TEMRA CD8 T cells
CCR7negCD45RApos/CD8pos (%)
CD27 neg TEMRA (%) |
68
53 |
43
12 |
35
4 |
12
1 |
| CD57 on CD8 T cells (%) |
50 (4-33) |
36 (4-33) |
6 (4-33) |
6 (4-33) |
| HLA-DR on TCRαβ (%) |
9 (1-4) |
2 (1-4) |
3 (1-4) |
4 (1-4) |
Memory B cells
CD27pos, IgDpos/CD19pos (%)
CD27pos, IgDneg/CD19 (%) |
3 (5-18)
2 (4-23)
|
2 (2.9-19)
6 (3.9-16.5)
|
6 (2.9-19)
6 (3.9-16.5)
|
10 (2.9-19)
22 (3.9-16.5
|
| Fraction of Somatic hypermutation (%) |
25 (27-88) |
43 (27-88) |
ND |
45 (27-88) |
T-cell proliferation (% among CD4)
PMA
Anti-CD3, Anti-CD2, Anti-CD28* |
49 (35-65)
94 (75-100)
|
24 (35-65)
18 (75-100)
|
24 (35-65)
86 (75-100)
|
67 (35-65)
94 (75-100)
|
