Table 2. Immune phenotype of patient 1, 2, and 3.
Patient 1 (6 yr) | Patient 2 (8 yr) | Patient 3 (2 yr) | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Relative counts | Reference values | Absolute | Reference values | Relative counts | Reference values | Absolute | Reference values | Relative counts | Reference values | Absolute | Reference values | |||
Lymphocytesa | 1,855 | 1,800–5,000 | 900 | 1,800–5,000 | 4,950 | 2,800–6,400 | ||||||||
B cellsa | 2.0 | 8.5–20.2 | 37 | 296–784 | 3.8 | 8.5–20.2 | 34 | 296–784 | 29.9 | 17.3–30.0 | 1,480 | 686–1,732 | ||
CD4a | 4.0 | 26.5–41.4 | 74 | 641–1,453 | 24.5 | 26.5–41.4 | 220 | 641–1,453 | 6.4 | 28.1–43.2 | 317 | 925–2,477 | ||
CD8b | 45.0 | 13–47 | 834 | 200–1,700 | 38.3 | 13–47 | 345 | 200–1,700 | 4.5 | 9–49 | 223 | 200–1,800 | ||
NK cellsb | 6.0 | 2–31 | 114 | 70–590 | 1.7 | 2–31 | 16 | 70–590 | 0.9 | 2–25 | 45 | 61–510 | ||
B cell subpopulationsa | ND | ND | ND | ND | ||||||||||
Transitional | 21.1 | 3.4–9.0 | 5 | 13–63 | ||||||||||
Naive | 68.7 | 47.8–69.8 | 23 | 154–413 | ||||||||||
IgM memory | 3.5 | 6.3–22 | 2 | 24–135 | ||||||||||
IgM only | 0.7 | 2–11.8 | 1 | 7–65 | ||||||||||
IgA cs memory | 0 | 1.1–6.1 | 0 | 5–35 | ||||||||||
IgG cs memory | 0 | 2.7–14 | 0 | 13–74 | ||||||||||
CD21low | 6.3 | |||||||||||||
CD4 subpopulationsa | ND | ND | ND | ND | ||||||||||
RTEc | 24.4 | 61–84.2 | 1 | |||||||||||
Naive | 2.4 | 55.6–75.8 | 5 | 375–1,096 | ||||||||||
CD45R0 | 97.5 | 24–43 | ||||||||||||
T reg cells | 2.0 | 2.3–7.7 | 5 | 18–86 | ||||||||||
cTFHd | 35.0 | 18.4–29.9 | 112 | 51–218 | ||||||||||
Th2+Th17-like cTFHd | 29.4 | 39.9–66.1 | 33 | 26–85 | ||||||||||
CD8 subpopulationsb | ND | ND | ND | ND | ||||||||||
Naive | 0.3 | 16–100 | 1 | 42–1,300 | ||||||||||
Central memory | 2.4 | 1–6 | 8 | 6–43 | ||||||||||
Effector memory | 65.9 | 5–100 | 227 | 45–410 | ||||||||||
Terminally differentiated | 1.6 | 15–41 | 6 | 57–340 | ||||||||||
γδ T cellse | ND | ND | 37.2 | <10 | 282 | 12–175 | 72 | <10 | 2,217 | 12–175 | ||||
DN T cellse | 51 | 38 | 72 | |||||||||||
NK T cellse | ND | ND | 0.005 | >0.01 | ND | ND | ND |
Relative counts are shown as a percentage of parental population. Absolute numbers refer to cells/microliter. CD4 T cell subpopulations: recent thymic emigrants (RTEs), CD31+ of CD45RA+ CD4 T cells; T reg cells, CD127− CD25hi of CD45RA− CD4 T cells; circulating T follicular helper–like cell (cTFH), CXCR5+ of CD45RA− CD4 T cells; Th2/Th17-like cTFH, CXCR3− of CXCR5+ CD45RA− CD4 T cells. CD8 T cell populations: naive, CCR7+ CD45RA+ CD27+; terminally differentiated, CCR7− CD45RA+ CD27−; central memory, CCR7+ CD45RA− CD27+; effector memory, CCR7− CD45RA− CD27−. Double-negative (DN) T cells, CD4−/CD8− of CD3 T cells. NK T cells, Vα24 Vβ11 CD3 T cells. cs, class switched.
Percentage of naive CD4 T cells.
Internal reference value.
Internal reference value of CD3 T cells.