Table 2 Immunophenotype of 4 patients with G6PC3 severe chronic neutropenia
UPN Age | 527 3 age 50 | Range of normal values for an adult* | 5805 age 3 | Range of normal values at age 3* | 6536 age 1 | Range of normal values at age 1* | 6262 age 6 | Range of normal values for at age 6* |
|---|---|---|---|---|---|---|---|---|
IgG/A/M in g/dL | 9.7 | 7.68-16.3 | 15.6 | 4.2-10.9 | 4.35. | 3.32-11.6 | 18.7 | 6.08-12.29 |
1.7 | 0.68-3.78 | 0.55 | 0.22-1.57 | 0.32 | 0.14-1.05 | 1.7 | 0.33-2 | |
1 | 0.60-2.63 | 0.7 | 0.45-2.63 | 0.34 | 0.45-1.90 | 1.7 | 0.46-1.97 | |
Antibodies against vaccine antigen | ++ | ++ | ++ | |||||
Lymphocytes | 700 | 1400–3300 | 831 | 2300–5400 | 3100 | 3600–8900 | 2800 | 2300–5400 |
CD3+ | 378 | 1200–2000 | 698 | 1400–3700 | 1891 | 2100–6200 | 1960 | 2100–6200 |
CD4+ | 252 | 530–1300 | 274.23 | 700–2200 | 1178 | 1300–3400 | 812 | 1300–3400 |
CD8+ | 98 | 330–920 | 332.4 | 490–1300 | 682 | 620–2000 | 756 | 490–1300 |
CD19+ | 49 | 110–570 | 58.17 | 390–1400 | 651 | 720–2600 | 364 | 390–1400 |
CD16+ CD56+ | 378 | 70–480 | 698.04 | 130–720 | 1891 | 180–920 | 1960 | 130–720 |
Distribution of naive/memory T cells | ||||||||
CD31+ CD45 + CD4+ (N 43–55%) | 2 | 25 | ||||||
CD45+CD4+ (N 43–55%) | 19 | 37 | 27 | 50 | ||||
CCR7 + CD45RA+/CD8+ (N 52–63%) | 5 | 43 | ||||||
CCR7 +CD45RA-/CD8+ (N 3–14%) | 7 | 6 | ||||||
CCR7 -CD45RA-/CD8+ (N 20–41%) | 78 | 35 | ||||||
CCR7 -CD45RA+/CD8+ (N 11–26%) | 10 | 16 | ||||||