Table 1.
Patient no. | Age (years) | Sex | EUROclass classification | Chronic diarrhoea | Splenomegaly | Granuloma | Bronchiectasis | Dose IgG/kg/week | IgG through level (g/l) | Leucocytes (103/μl) | Lymphocytes (%) | Lymphocytes (103/μl) | CD3+ (103/μl) | CD3+CD4+ (103/μl) | CD3+CD8+ (103/μl) | CD19+ (103/μl) | NK cells (103/μl) | CD4+CD45RO+/CD4+(%) |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 33 | F | smB-21lo | Yes | Yes | Yes | No | 0.12 | 4.2 | 5.2 | 9 | 0.47 | 0.37 | 0.23 | 0.12 | 0.05 | 0.04 | 87 |
2 | 78 | F | smB-21lo | No | Yes | No | Yes | 0.12 | 6.66 | 5.1 | 36 | 1.84 | 1.36 | 0.55 | 0.66 | 0.39 | 0.07 | 86 |
3 | 59 | F | smB-21lo | No | Yes | No | Yes | 0.12 | 8.3 | 3.3 | 28 | 0.92 | 0.72 | 0.37 | 0.34 | 0.13 | 0.06 | 93 |
4 | 45 | F | smB-21lo | No | Yes | No | Yes | 0.08 | 5.32 | 8.3 | 25 | 2.08 | 1.58 | 0.87 | 0.62 | 0.39 | 0.08 | 80 |
5 | 68 | F | smB-21lo | Yes | Yes | No | Yes | 0.09 | 5.95 | 4.2 | 27 | 1.13 | 0.90 | 0.43 | 0.43 | 0.09 | 0.15 | 78 |
6 | 35 | F | smB-21norm | No | No | No | No | 0.10 | 6.95 | 5.9 | 25 | 1.48 | 1.19 | 0.65 | 0.52 | 0.04 | 0.24 | 47 |
7 | 40 | F | ND | No | Yes | No | No | 0.07 | 5.63 | 8.7 | 18 | 1.57 | 1.35 | 0.53 | 0.83 | ND | 0.22 | 85 |
8 | 22 | F | smB-21lo | No | Yes | No | Yes | 0.06 | 7.66 | 4.2 | 44 | 1.85 | 1.48 | 0.79 | 0.61 | 0.24 | 0.11 | 55 |
9 | 65 | F | smB-21lo | No | No | No | No | 0.10 | 6.61 | 7.4 | 21 | 1.55 | 0.93 | 0.56 | 0.31 | 0.30 | 0.30 | 70 |
10 | 55 | F | smB-21lo | No | No | No | Yes | 0.06 | 5.78 | 5.7 | 29 | 1.65 | 1.19 | 0.58 | 0.36 | 0.20 | 0.26 | 88 |
11 | 19 | M | smB-21norm | No | No | No | No | 0.09 | 5.2 | 4.4 | 25 | 1.10 | 0.84 | 0.39 | 0.39 | 0.17 | 0.10 | 70 |
12 | 58 | F | smB-21lo | Yes | Yes | No | Yes | 0.08 | 6.23 | 5.7 | 23 | 1.31 | 0.88 | 0.43 | 0.41 | 0.18 | 0.25 | 92 |
13 | 52 | M | ND | Yes | Yes | Yes | Yes | 0.10 | 6.37 | 15.4 | 4 | 0.62 | 0.49 | 0.26 | 0.19 | ND | 0.11 | 70 |
14 | 25 | M | smb+21lo | No | No | No | No | NT | 3.51 | 7.8 | 24 | 1.87 | 1.07 | 0.58 | 0.37 | 0.24 | 0.54 | 73 |
15 | 30 | F | smB-21lo | Yes | No | No | Yes | 0.07 | 5.56 | 2.7 | 30 | 0.81 | 0.76 | 0.58 | 0.18 | 0.02 | 0.02 | 36 |
16 | 51 | M | smB-21lo | Yes | Yes | No | Yes | 0.09 | 3.1 | 4.4 | 35 | 1.54 | 1.43 | 0.83 | 0.57 | 0.05 | 0.06 | 98 |
17 | 45 | F | smB-21lo | No | Yes | No | No | 0.10 | 3.07 | 6.4 | 12 | 0.77 | 0.62 | 0.25 | 0.37 | 0.07 | 0.07 | 85 |
18 | 29 | F | smB-21norm | No | No | No | Yes | NT | 2.35 | 7.0 | 24 | 1.68 | 0.99 | 0.60 | 0.32 | 0.55 | 0.12 | 68 |
19 | 35 | M | smB-21norm | No | Yes | No | No | 0.08 | 5.2 | 5.6 | 31 | 1.74 | 1.48 | 0.49 | 0.95 | 0.17 | 0.09 | 60 |
20 | 41 | F | smB-21lo | No | Yes | No | Yes | 0.11 | 6.33 | 7.6 | 28 | 2.13 | 1.66 | 0.83 | 0.66 | 0.32 | 0.13 | 58 |
21 | 58 | M | smB-21lo | Yes | Yes | No | Yes | 0.09 | 5.22 | 9.2 | 24 | 2.21 | 1.19 | 0.66 | 0.49 | 0.77 | 0.22 | 68 |
22 | 45 | F | smB-21lo | No | No | No | Yes | 0.11 | 6.06 | 5.6 | 45 | 2.52 | 2.24 | 0.86 | 1.21 | 0.20 | 0.03 | 75 |
23 | 51 | F | ND | No | Yes | No | Yes | 0.11 | 4.83 | 10.3 | 17 | 1.75 | 1.66 | 0.84 | 0.81 | ND | 0.09 | 91 |
24 | 72 | F | smb+21lo | No | Yes | No | No | 0.06 | 4.9 | 8.3 | 38 | 3.15 | 2.40 | 1.58 | 0.91 | 0.69 | 0.03 | 58 |
25 | 21 | F | smB-21norm | No | Yes | Yes | Yes | 0.08 | 6.4 | 4.4 | 18 | 0.79 | 0.62 | 0.38 | 0.20 | 0.13 | 0.02 | 86 |
26 | 42 | F | smB+21norm | No | No | No | No | 0.07 | 4.7 | 5.7 | 21 | 1.20 | 0.83 | 0.57 | 0.25 | 0.22 | 0.16 | 92 |
27 | 38 | M | smB-21lo | No | Yes | Yes | No | 0.09 | 5.73 | 5.2 | 18 | 0.94 | 0.85 | 0.43 | 0.42 | 0.03 | 0.03 | 88 |
28 | 51 | F | smB-21lo | No | Yes | No | Yes | 0.14 | 6.12 | 3.3 | 19 | 0.63 | 0.57 | 0.39 | 0.17 | 0.02 | 0.04 | 98 |
29 | 60 | M | smB-21norm | Yes | Yes | No | No | 0.06 | 6.26 | 4.9 | 19 | 0.93 | 0.59 | 0.42 | 0.13 | 0.19 | 0.14 | 75 |
30 | 41 | F | smB-21lo | Yes | Yes | No | Yes | 0.04 | 2.96 | 3.8 | 29 | 1.10 | 0.94 | 0.37 | 0.53 | 0.12 | 0.04 | 82 |
31 | 23 | F | smB-21lo | No | Yes | No | No | 0.06 | 4.5 | 9.0 | 31 | 2.79 | 2.32 | 1.42 | 0.67 | 0.33 | 0.11 | 52 |
32 | 51 | M | smB-21lo | No | No | No | Yes | 0.10 | 5.3 | 5.2 | 31 | 1.61 | 1.26 | 0.37 | 0.77 | 0.13 | 0.23 | 77 |
33 | 70 | M | smB-21lo | No | Yes | Yes | No | 0.05 | 4.49 | 4.7 | 18 | 0.85 | 0.76 | 0.43 | 0.36 | 0.02 | 0.06 | 98 |
34 | 29 | F | smB-21lo | Yes | Yes | No | No | 0.08 | 5.8 | 4.1 | 27 | 1.11 | 0.95 | 0.61 | 0.30 | 0.10 | 0.06 | 63 |
35 | 22 | M | smB-21lo | Yes | Yes | No | No | 0.07 | 4.43 | 6.1 | 37 | 2.26 | 1.31 | 0.50 | 0.84 | 0.23 | 0.70 | 81 |
NT: not treated; ND: not determined, the patient had B cells < 1% of total lymphocytes; F: female; M: male.