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. 2016 Jun 27;213(7):1185–1199. doi: 10.1084/jem.20151110

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.

c

Percentage of naive CD4 T cells.

d

Internal reference value.

e

Internal reference value of CD3 T cells.