Table 2.
Immunological parameters
| Pat. 1 | Pat. 2 | Pat. 3 | ||
|---|---|---|---|---|
| Autoantibodies | ANA, pANCA, PR3 | cANCA, anti-cardiolipin, B2GLG | cANCA, PL, anti-cardiolipin, PT | |
| Age at immunoglobulins and lymphocyte subset analysis (years) | 12 | 8 | 1 year and 10 months | |
| Total immunoglobulins | IgA mg/dl | 274 (n.v. 70–400) | 340 (n.v. 34–305) | 130 (n.v. 36–165) |
| IgG mg/dl | 1631 (n.v. 700–1600) | 1639 (n.v. 500–1560) | 1674 (n.v. 520–1400) | |
| IgM mg/dl | 195.0 (n.v. 40–230) | 138 (n.v. 25–210) | 266 (n.v. 35–104) | |
| IgE (KU/l) |
855 (n.v. < 200) |
89,7 (n.v. < 90) |
14,8 (n.v. < 40) |
|
| CD3 (a.n.) |
1008 (n.v. 700–3200) |
1039 (n.v. 700–3200) |
2953 (n.v. 2100–6200) |
|
| CD4 (a.n.) | 645 (n.v. 300–2000) | 742 (n.v. 300–2400) | 1988 (n.v. 1300–3400) | |
| CD8 (a.n.) | 337 (n.v. 300–1800) | 313 (n.v. 300–1800) | 812 (n.v. 620–2000) | |
| CD19 (a.n.) | 238 (n.v. 200–1600) | 208 (n.v. 100–1200) | 1647 (n.v. 720–2600) | |
| CD3-CD56+ (a.n.) | 54 (n.v. 90–900) | 346 (n.v. 90–1000) | 994 (n.v. 180–920) | |
| CD4 CD45RA+ (% of CD4+) | 73 (n.v. 46–77) | 92 (n.v. 46–77) | 85 (n.v. 63–91) | |
| CD4 CD45RO+ (% of CD4+) | 27 (n.v. 13–30) | 8 (n.v. 13–30) | 15 (n.v. 7–20) | |
| CD8 CD45RA+ (% of CD8+) | NA | 93 (n.v. 63–92) | 70 (n.v. 71–98) | |
| CD8 CD45RO+ (% of CD8+) | NA | 7 | 30 | |
| Lymphocyte proliferation | Normal in response to PHA | Normal in response to PHA | Normal in response to PHA, defective to OKT3 | |
| Vaccinations | DtP, Polio, Hib, hepatitis B, MMR (1 dose) | DtP, Polio, Hib, hepatitis B, MMR | Not performed | |
| Response to vaccine (years of age when tested) | Nonprotective to tetanus toxoid and diphtheria (9) | Protective to tetanus toxoid (8) | Not tested |