Table 1 Immunological data of the patients showing decrease level of IgA, T and B lymphopenia; reduced naïve T subsets (CD4RA,CD8RA); low lymphocyte proliferation to PHA; nonprotective specific antibodies response against tetanus, after vaccination; skewed T Cell Receptor repertoire
WBC/ml | 9080 | |
|---|---|---|
Serum immunoglobulin mg/dl | ||
IgA | <5 | |
IgM | 187 | |
IgG | 1158 | |
IgG1 | 906 | |
IgG2 | 94.3 | |
IgG3 | 10:01 | |
IgG4 | 7.16 | |
Lymphocyte phenotype (percentage and absolute number) | ||
Absolute count/ml | 1260 | |
CD3 | 33% | 534.6 |
CD4 | 18% | 291.6 |
CD4RA | 1.6% | 25.9 |
CD4RO | 15.5% | 251.1 |
CD8 | 11% | 178.2 |
CD8RA | 5.6% | 90.7 |
CD8RO | 5.2% | 84.2 |
CD19 | 6% | 97.2 |
CD16-56 | 50% | 810 |
Response to mitogens (counts per min) | ||
PHA | 19% | |
OKT3 | 48% | |
Response to vaccination | ||
Ab anti-Tet | 0.1U/ml | |
Ab anti-PcP | 50 mg/l | |
Ab anti-HiB | 0.3 mg/l | |
Phenotype B | ||
CD22+CD27+IgD+IgM+ | 7.2% | |
CD22+CD27+IgD-IgM- | 20.2% | |
CD22+CD27-IgD+IgM+ | 60.5% | |
CD21lowCD38NEG | 28.7% | |
CD4+ and CD8+ TCR analysis (Spectratyping) | ||
CD4+ SKEWED | 43% | |
CD8+ SKEWED | 100% |