Table 1 Clinical characteristics of our patients with X-linked agammaglobulinemia
Index case | Brother | Reference range | |
|---|---|---|---|
Age years | 6 | 8 | |
WBC × 1000/ul | 6.5 | 6.7 | 6.0-10.4 |
Seg % | 58 | 38 | 27.8-57.6 |
Lymph % | 36 | 47 | 34.4-62.8 |
Mono % | 3 | 8 | 2.0-7.6 |
Eosinophil % | 1 | 5 | 0-6.8 |
IgG mg/dl | 823 | 963 | 608-1572 |
IgA mg/dl | 129 | 267 | 33-236 |
IgM mg/dl | <4.17 | 11.1 | 48-242 |
IgE IU/ml | 184 | 2160 | Child (3–9 y/o) : < 53 IU/mL |
C3 mg/dl | 48.2 | 66.9 | 77-195 |
C4 mg/dl | 12.5 | 16.5 | 7-40 |
CH50 CAE unit | 46.5 | 71.97 | 63-145 |
ANA | 1:40 | <1:40 | |
Anti-dsDNA IU/ml | 0.7 | <10, negative | |
Anti-Ro U/ml | 3.2 | <7, negative | |
Anti-La U/ml | 0.3 | <7, negative | |
Anti-Sm U/ml | 0.1 | <5, negative | |
Anti-nRNP U/ml | 0.8 | <5, negative | |
Antistreptolysin-O IU/ml | <25.0 | <100 IU | |
Urine protein | 2+ | - | Negative |
Urine OB | 3+ | - | Negative |
Serum Creatinine mg/dl | 0.4 | 0.42 | 0.2-1.0 |
CD3 % | 82 | 94.8 | Child (>2 y/o) : 58-87 |
DR+/CD3 + % | 12 | 15.5 | |
CD19 % | 1 | 0.8 | Child (>2 y/o) : 5-23 |
CD4 % | 44 | 35.9 | Child (>2 y/o) : 32-62 |
T-cell subsets CD8 % | 37 | 49.3 | Child (>2 y/o) : 12-45 |
HLA-DR positive % | 18 | 16.8 |