Table I.
Phenotype of patients
| Characteristic | P1 | P2a | P3a | P4 | P5 | Age-matched controls |
|---|---|---|---|---|---|---|
| Age at diagnosis (yr) | 6 | 6 | 8 | 4 | 4 | — |
| Serum Ig levels (g/l) | — | |||||
| IgM | 16 | 4.1 | 2.6 | 2.8 | 0.9 | 0.5–1.2 |
| IgG | 0.6 | 3.6 | 5.7 | 0.7 | 1.4 | 6.8–12.5 |
| IgA | 0.3 | <0.03 | <0.03 | <0.03 | <0.03 | 0.6–1.6 |
| T lymphocyte counts/μl | 2,400 | 1,420 | 1,238 | 1,612 | 1,300 | 1,200–2,600 |
| B lymphocyte counts/μl | 452 | 120 | 170 | 462 | 440 | 110–570 |
| IgD+/CD19+ (%)b | 99.9 | 99.2 | ND | 99.8 | ND | 83–93 |
| CD27+/CD19+ (%)b | 1.5 | 3.3 | 3.1 | 4.7 | 3.1 | 13–58 |
a
P2 and P3 are siblings.
b
Gated on CD19+ B cells.