Immune function studies
| . | Sib A . | Sib B . | Patient Y . | Patient R . |
|---|---|---|---|---|
| Age at diagnosis, y | 4 | 16 | 5.5 | 1.3 |
| WBC, 109/L | 6.16 | 6.82 | 3.43 | 7.15 |
| Total lymphs | 1 060 | 1 570 | 274 | 787 |
| (2 000-8 000) | (1 000-4 800) | (2 000-8 000) | (4 000-10 500) | |
| CD3 | 974 | 1 522 | 181 | 449 |
| (1 600-2 700) | (782-2 834) | (1 600-2 700) | (2 200-4 100) | |
| CD4 | 115 | 235 | 110 | 181 |
| (1 000-1 700) | (322-1 750) | (1 000-1 700) | (1 400-2 800) | |
| CD8 | 827 | 1 130 | 82 | 244 |
| (600-1 000) | (338-1 086) | (600-1 000) | (800-1 800) | |
| CD19 | 21 | 31 | 8 | 71 |
| (400-800) | (67-555) | (400-800) | (700-1 600) | |
| CD16/56 | 31 | < 16 | 37 | 110 |
| (200-400) | (200-400) | (200-400) | (200-600) | |
| PHA* | 23 781/24% | 80 884/67% | ||
| Con A* | 18 369/27% | 68 741/75% | ||
| Allogeneic cells* | 15 954/36% | 41 209/81% | ||
| IgG, g/L | 13.7 | 11.2 | 12.3 | 12.3 |
| (4.6-12.4) | (6.4-13.5) | (4.6-12.4) | (3.5-12.1) | |
| IgA, g/L | 1.4 | 1.5 | 1.5 | 0.9 |
| (0.24-1.5) | (0.7-3.1) | (0.24-1.5) | (0.14-1.06) | |
| IgM, g/L | 1.05 | 1.13 | 0.62 | 1.95 |
| (0.43-1.96) | (0.56-3.52) | (0.43-1.96) | (0.43-1.73) | |
| IgE, IU/L | 3 820 | 347 | 8 580 | |
| (8-250) | (5-500) | (1.6-30) | ||
| Tetanus antibody | 2.07 | 10.4 | 2.8 | 10.8 |
| Pneumococcus antibody | 8.06 | 9.5 |
| . | Sib A . | Sib B . | Patient Y . | Patient R . |
|---|---|---|---|---|
| Age at diagnosis, y | 4 | 16 | 5.5 | 1.3 |
| WBC, 109/L | 6.16 | 6.82 | 3.43 | 7.15 |
| Total lymphs | 1 060 | 1 570 | 274 | 787 |
| (2 000-8 000) | (1 000-4 800) | (2 000-8 000) | (4 000-10 500) | |
| CD3 | 974 | 1 522 | 181 | 449 |
| (1 600-2 700) | (782-2 834) | (1 600-2 700) | (2 200-4 100) | |
| CD4 | 115 | 235 | 110 | 181 |
| (1 000-1 700) | (322-1 750) | (1 000-1 700) | (1 400-2 800) | |
| CD8 | 827 | 1 130 | 82 | 244 |
| (600-1 000) | (338-1 086) | (600-1 000) | (800-1 800) | |
| CD19 | 21 | 31 | 8 | 71 |
| (400-800) | (67-555) | (400-800) | (700-1 600) | |
| CD16/56 | 31 | < 16 | 37 | 110 |
| (200-400) | (200-400) | (200-400) | (200-600) | |
| PHA* | 23 781/24% | 80 884/67% | ||
| Con A* | 18 369/27% | 68 741/75% | ||
| Allogeneic cells* | 15 954/36% | 41 209/81% | ||
| IgG, g/L | 13.7 | 11.2 | 12.3 | 12.3 |
| (4.6-12.4) | (6.4-13.5) | (4.6-12.4) | (3.5-12.1) | |
| IgA, g/L | 1.4 | 1.5 | 1.5 | 0.9 |
| (0.24-1.5) | (0.7-3.1) | (0.24-1.5) | (0.14-1.06) | |
| IgM, g/L | 1.05 | 1.13 | 0.62 | 1.95 |
| (0.43-1.96) | (0.56-3.52) | (0.43-1.96) | (0.43-1.73) | |
| IgE, IU/L | 3 820 | 347 | 8 580 | |
| (8-250) | (5-500) | (1.6-30) | ||
| Tetanus antibody | 2.07 | 10.4 | 2.8 | 10.8 |
| Pneumococcus antibody | 8.06 | 9.5 |
Lymphocyte counts are expressed as 106 cells/L. Normal ranges for age are shown in parentheses.
WBC indicates white blood cell; PHA, phytohemagglutinin; and Con A, concanavalin A.
cpm/% response (patient cpm, mitogen-patient cpm, media)/(control cpm, mitogen-control cpm, media) × 100.