Table 1.
Age range and median age at last news from patient in years of the 12 most prevalent PID diagnoses and WAS, XLP, and FHLH (patients with no HSCT or gene therapy), living patients.
12 PIDs, WAS, XLP, FHLH | Male (n) | Female (n) | Total (no HSCT/no gene therapy) | Age range (years) | Median age (years) | Living patients with and withoutHSCT/gene therapy | |
---|---|---|---|---|---|---|---|
CVID | 313 | 352 | 665 | 99.8% | 3–88 | 40 | 666 |
Unclassified antibody deficiency | 102 | 119 | 221 | 99.1% | 0–80 | 28 | 223 |
CGD | 46 | 12 | 58 | 51% | 0–59 | 21 | 114 |
Agammaglobulinemia | 90 | 7 | 97 | 98% | 2–54 | 17 | 99 |
Isolated IgG subclass deficiency | 40 | 42 | 82 | 100% | 4–68 | 39 | 82 |
SCID | 6 | 2 | 8 | 11% | 0–21 | 15 | 76 |
Unclassified IDs | 22 | 20 | 42 | 93% | 3–79 | 20 | 45 |
Combined ID | 21 | 17 | 38 | 60% | 3–57 | 18 | 63 |
DGS | 25 | 27 | 52 | 100% | 0–40 | 8 | 52 |
Selective IgA deficiency | 17 | 30 | 47 | 100% | 3–75 | 23 | 47 |
A-T | 30 | 25 | 55 | 96% | 3–37 | 13 | 57 |
HIES | 28 | 19 | 47 | 85% | 0–43 | 16 | 55 |
WAS | 7 | 0 | 7 | 25% | 0–31 | 20 | 28 |
XLP | 14 | 1 | 15 | 68% | 7–52 | 19 | 22 |
FHLH | 3 | 0 | 3 | 17% | 2–13 | 7 | 18 |
Total | 763 | 672 | 1,437 | 87% | 0–88 | 1,647 |