Table 2 Evolution of the immune and autoimmune status of the proposita
From: Rituximab resistant evans syndrome and autoimmunity in Schimke immuno-osseous dysplasia
Test results at the indicated ages | |||
|---|---|---|---|
Age (years) | 3§ | 4 | 5¶ |
Test (normal values) | |||
IgG (g/l) (5.53-10.20) | 7.72 | 12.1 | 1.63 |
IgA (g/l) (0.33-0.91) | 0.83 | 1.66 | 0.43 |
IgM (g/l) (0.47-1.67) | 0.97 | 2.19 | 3.49 |
C3 (g/l) (0.83-2.25) | 1.06 | 1.47 | 1.41 |
C4 (g/l) (0.14-0.35) | 0.18 | 0.10 | 0.11 |
ANA | negative | negative | negative |
ANCA | negative | negative | negative |
ACLA (GPL/ml) (0.8-11) | 7.0 | 6.3 | 4.3 |
ENA screening | negative | negative | NM |
ATA IgA (U/ml) (0-10) | 2.09 | 2.11 | NM |
CD4 absolute count x103/ml (35-51%) | 130 (12%) | 200 (13%) | 70 (50%) |
CD8 absolute count x103/ml (18-40%) | 20 (1.8%) | 170 (11%) | 60 (44%) |
CD4/CD8 (1-3%) | 6.7% | 1.2% | 1.1% |
CD19 absolute count x103/ml (9-35%) | 0.440 (45%) | 530 (35%) | 0 (0%)* |