Table 3.
Lymphocyte Counts in Individuals with EXTL3 Mutations and Pediatric Control Individuals
| Individual | Age | CD3+T Cell (per μL)a | CD3+CD4+T Cells (per μL) | CD3+CD8+T Cells (per μL) | CD19+B Cells (per μL) | CD56+NK Cells (per μL) | IgG (g/L) | IgA (g/L) | IgM (g/L) |
|---|---|---|---|---|---|---|---|---|---|
| A:II-1 | NA | 0 | 0 | 0 | 1436 | 279 | 1.0 | <0.1 | 0.3 |
| B:II-1 | 7 years | 367 (25%) | 157 (8%) | 210 (11%) | 656 (34%) | 262 (13%) | 5.65 | 0.39 | 0.24 |
| 8 years | 488 (25%) | 176 (9%) | 293 (15%) | 819 (42%) | 585 (30%) | 4.52 | 0.29 | <0.2 | |
| B:II-2b | 3 months | 2442 (51%) | 2442 (51%) | 0 | 1436 (30%) | 575 (12%) | 12.9 | 0.2 | 2.5 |
| C:II-1 | 12 years | 819 (44%) | 348 (19%) | 221 (12%) | 557 (30%) | 453 (25%) | NA | NA | NA |
| D:III-1 | NA | NA | NA | NA | NA | NA | NA | NA | NA |
| D:III-2 | NA | NA | NA | NA | NA | NA | NA | NA | NA |
| D:IV-1 | 12 years | 600 (36%) | 382 (23%) | 206 (12%) | 538 (32%) | 513 (31%) | NA | NA | NA |
| E:II-1c | 2.5 months | 1800 (40%) | 500 (11%) | 1310 (25%) | 2030 (45%) | 360 (8%) | <1.6 | <0.4 | 0.5 |
| E:II-2 | 9 months | 10 (0.2%) | 0 | 0 | 1950 (69%) | 760 (27%) | 4.7 | <0.06 | 0.11 |
| Pediatric control individuals | <2 years | 700–1,500 | 400–900 | 400–1,100 | 300–1,500 | 50–400 | 5–10 | 0.7–1.5 | 0.3–1.2 |
Parentheses show the percentage of the lymphocyte subtype within the total lymphocyte count. NA means no data available.
a
All values were obtained before hematopoietic stem cell transplantation (HSCT).
b
IgG before administration of intravenous immunoglobulins and before HSCT. Ig subclasses (g/L): IgG1, 11.9; IgG2, 1.69; IgG3, 0.57; IgG4, 0.19.
c
Already developed Omenn syndrome.