Flow cytometric phenotype analysis of patient PBMCs
| Marker | P2 | P3 | P4 | Normal Ctrl | ||||
| % | Abs # | % | Abs # | % | Abs # | % | Abs # | |
| B cell lineages | ||||||||
| CD19+ B | 64.9 | 4,703 | 62.8 | 4,710 | 52.7 | 8,105 | 14–33 | 390–1,400 |
| CD20+IgD+IgM+ | 64.2 | 4,658 | 62.3 | 4,673 | 51.7 | 7,951 | 7.4–12.1 | 50–276 |
| CD20+CD10+ | 31.5 | 2,286 | 34.1 | 2,558 | 26.7 | 4,100 | (0.1–3.4) | (2–76) |
| CD20+CD27+ | 1 | 76 | 0.9 | 67 | 0.4 | 62 | 2.9–7.3 | 12–68 |
| CD20+IgG+ | 0.1 | 9 | 0.1 | 9 | 0.1 | 15 | (0.2–2.2) | (4–49) |
| CD20+IgA+ | 0.1 | 5 | 0 | 3 | ND | ND | (0.1–1.3) | (3–9) |
| CD20+CD5+ | 43.6 | 3,164 | 43.4 | 3,255 | 35.7 | 5,491 | (0.7–9.7) | (14–159) |
| T and NK cell lineages | ||||||||
| CD3+ T | 31.5 | 2,287 | 32.9 | 2,464 | 40.5 | 6,229 | 56–75 | 1,400–3,700 |
| CD4+ T | 16.6 | 1,202 | 17.1 | 1,282 | 9.0 | 1,384 | 28–47 | 700–2,200 |
| CD4+CD45RA+ | 10.5 | 760 | 16.6 | 1,245 | 2.5 | 385 | 20–33 | 430–1,500 |
| CD4+CD45RO+ | 5.6 | 405 | 0 | 2 | 1.4 | 215 | 6–17 | 220–660 |
| CD8+ T | 9.6 | 697 | 11.8 | 885 | 27.5 | 4,230 | 16–30 | 490–1,300 |
| CD8+CD45RA+ | 8.4 | 612 | 11.2 | 837 | 7.1 | 1,092 | 16–22 | 380–1,100 |
| CD8+CD45RO+ | 0.5 | 39 | 0.1 | 8 | 5.0 | 769 | 3–11 | 90–440 |
| NK cells | 3.7 | 268 | 4.3 | 325 | 6.9 | 1,061 | 4–17 | 130–720 |
| TCR-α/β+CD4−CD8− | 5.0 | 364 | 3.6 | 272 | 3.9 | 600 | (0.3–1.3) | (6–23) |
| CD4+CD25+Foxp3+ | 1.1 | 83 | 0.6 | 33 | 0.3 | 40 | (1.6–4.3) | (25–89) |
| Marker | P2 | P3 | P4 | Normal Ctrl | ||||
| % | Abs # | % | Abs # | % | Abs # | % | Abs # | |
| B cell lineages | ||||||||
| CD19+ B | 64.9 | 4,703 | 62.8 | 4,710 | 52.7 | 8,105 | 14–33 | 390–1,400 |
| CD20+IgD+IgM+ | 64.2 | 4,658 | 62.3 | 4,673 | 51.7 | 7,951 | 7.4–12.1 | 50–276 |
| CD20+CD10+ | 31.5 | 2,286 | 34.1 | 2,558 | 26.7 | 4,100 | (0.1–3.4) | (2–76) |
| CD20+CD27+ | 1 | 76 | 0.9 | 67 | 0.4 | 62 | 2.9–7.3 | 12–68 |
| CD20+IgG+ | 0.1 | 9 | 0.1 | 9 | 0.1 | 15 | (0.2–2.2) | (4–49) |
| CD20+IgA+ | 0.1 | 5 | 0 | 3 | ND | ND | (0.1–1.3) | (3–9) |
| CD20+CD5+ | 43.6 | 3,164 | 43.4 | 3,255 | 35.7 | 5,491 | (0.7–9.7) | (14–159) |
| T and NK cell lineages | ||||||||
| CD3+ T | 31.5 | 2,287 | 32.9 | 2,464 | 40.5 | 6,229 | 56–75 | 1,400–3,700 |
| CD4+ T | 16.6 | 1,202 | 17.1 | 1,282 | 9.0 | 1,384 | 28–47 | 700–2,200 |
| CD4+CD45RA+ | 10.5 | 760 | 16.6 | 1,245 | 2.5 | 385 | 20–33 | 430–1,500 |
| CD4+CD45RO+ | 5.6 | 405 | 0 | 2 | 1.4 | 215 | 6–17 | 220–660 |
| CD8+ T | 9.6 | 697 | 11.8 | 885 | 27.5 | 4,230 | 16–30 | 490–1,300 |
| CD8+CD45RA+ | 8.4 | 612 | 11.2 | 837 | 7.1 | 1,092 | 16–22 | 380–1,100 |
| CD8+CD45RO+ | 0.5 | 39 | 0.1 | 8 | 5.0 | 769 | 3–11 | 90–440 |
| NK cells | 3.7 | 268 | 4.3 | 325 | 6.9 | 1,061 | 4–17 | 130–720 |
| TCR-α/β+CD4−CD8− | 5.0 | 364 | 3.6 | 272 | 3.9 | 600 | (0.3–1.3) | (6–23) |
| CD4+CD25+Foxp3+ | 1.1 | 83 | 0.6 | 33 | 0.3 | 40 | (1.6–4.3) | (25–89) |
Percentages and absolute cell counts (per microliter of whole blood) of each cell population. Ranges in age-matched pediatric controls are listed on the right, based on published reports (Shearer et al., 2003; Piątosa et al., 2010). Parentheses indicate normal adult controls for which pediatric data were not readily available. Patient ages (years) at time of phenotyping were 8 (P2), 6 (P3), and 6 (P4).