Hematological and immunological features of the patient
| | | Patient | Reference values | |
|---|---|---|---|---|
| Age at evaluation (years) | 7 | 12 | 14 | |
| Serum Ig levels | ||||
| IgG* (g/l) | 5.8 | 6.24 | 11.43 | 6.98–15.6 |
| IgA (g/l) | 0.89 | 1.35 | 1.46 | 0.53–2.9 |
| IgM (g/l) | 0.52 | 0.67 | 0.72 | 0.31–1.62 |
| Blood parameters | ||||
| Hemoglobin (×109/liter) | | 14.2 | 14.9 | 13–17 |
| MCV (fl) | | NA | 88.4 | 78–96 |
| Leukocytes (×109/liter) | | 7.15 | 7.58 | 4.5–13 |
| Platelets (×109/liter) | | 315 | 291 | 150–450 |
| Monocytes (×109/liter) | | NA | 0.75 | 0.3–1 |
| Neutrophils (×109/liter) | | 3.97 | 4.28 | 2–8 |
| T lymphocytes | ||||
| CD3+(/μl) | | 2,534 | 1,729 | 1,000–2,200 |
| CD3+CD4+(/μl) | | 1,404 | 922 | 530–1,300 |
| Naive CD4+/CD45RA+ (%) | | 71 | 66.4 | 58–70 |
| Memory CD4+/CD45RO+ (%) | | 29 | 33.6 | 34–67 |
| CD4+/CD31+CD45RA+ (%) | | 49 | 49.6 | 43–55 |
| TH1 CD4+CD45RA−/CXCR3+CXCR5− (%) | | NA | 61 | 35.3–61 |
| TH2 CD4+CD54RA−/CCR4+CCR6− (%) | | NA | 7.5 | 4.4–13 |
| TH17 CD4+CD45RA−/CCR4+CCR6+ (%) | | NA | 9.8 | 5.5–16.5 |
| Follicular helper CD4+CD45RA−/CXCR5+ (%) | | NA | 15.6 | 16.3–30.2 |
| Treg CD4+/CD25+CD127LOW (%) | | NA | 9.5 | 4.2–10.5 |
| CD8+ (/μl) | | 924 | 624 | 330–920 |
| Naive CD8+/CCR7+CD45RA+ (%) | | 77 | 82 | 40–75 |
| Central memory CD8+/CCR7+CD45RA− (%) | | 3 | 1.4 | 2–7 |
| Effector memory CD8+/CCR7−CD45RA− (%) | | 12 | 8.1 | 11–20 |
| Terminal effector CD8+/CCR7−CD45RA+ (%) | | 8 | 8.5 | 7–32 |
| NK cells | ||||
| CD16+CD56+ (/μl) | | 342 | 149 | 70–480 |
| B lymphocytes | ||||
| CD19+ (/μl) | | 548 | 478 | 193–628 |
| Naive B cells (CD27−IgD+) (%) | | 92 | 92 | 61.6–87.4 |
| Memory B cells (CD27+) (%) | | 3 | 7.6 | 7–29 |
| Unswitched memory CD27+IgD+ (%) | | 2 | 5.7 | 2.6–13.4 |
| Switched memory CD27+IgD− (%) | | 2 | 1.6 | 4–21.2 |
| Transitional CD24++CD38++ (%) | | 9 | 4.9 | 3.9–7.8 |
| Plasmablast CD24−CD38++ (%) | | 0.2 | 0 | 0.3–1.7 |
| CD19+ CD21−CD38− (%) | | 1 | 0.8 | 0.9–3.3 |
| | | Patient | Reference values | |
|---|---|---|---|---|
| Age at evaluation (years) | 7 | 12 | 14 | |
| IgG* (g/l) | 11.43 | 6.98–15.6 | ||
| IgA (g/l) | 0.89 | 1.35 | 1.46 | 0.53–2.9 |
| IgM (g/l) | 0.52 | 0.67 | 0.72 | 0.31–1.62 |
| Hemoglobin (×109/liter) | | 14.2 | 14.9 | 13–17 |
| MCV (fl) | | NA | 88.4 | 78–96 |
| Leukocytes (×109/liter) | | 7.15 | 7.58 | 4.5–13 |
| Platelets (×109/liter) | | 315 | 291 | 150–450 |
| Monocytes (×109/liter) | | NA | 0.75 | 0.3–1 |
| Neutrophils (×109/liter) | | 3.97 | 4.28 | 2–8 |
| CD3+(/μl) | | 1,729 | 1,000–2,200 | |
| CD3+CD4+(/μl) | | 922 | 530–1,300 | |
| Naive CD4+/CD45RA+ (%) | | 66.4 | 58–70 | |
| Memory CD4+/CD45RO+ (%) | | 34–67 | ||
| CD4+/CD31+CD45RA+ (%) | | 49 | 49.6 | 43–55 |
| TH1 CD4+CD45RA−/CXCR3+CXCR5− (%) | | NA | 61 | 35.3–61 |
| TH2 CD4+CD54RA−/CCR4+CCR6− (%) | | NA | 7.5 | 4.4–13 |
| TH17 CD4+CD45RA−/CCR4+CCR6+ (%) | | NA | 9.8 | 5.5–16.5 |
| Follicular helper CD4+CD45RA−/CXCR5+ (%) | | NA | 16.3–30.2 | |
| Treg CD4+/CD25+CD127LOW (%) | | NA | 9.5 | 4.2–10.5 |
| CD8+ (/μl) | | 624 | 330–920 | |
| Naive CD8+/CCR7+CD45RA+ (%) | | 40–75 | ||
| Central memory CD8+/CCR7+CD45RA− (%) | | 3 | 2–7 | |
| Effector memory CD8+/CCR7−CD45RA− (%) | | 12 | 11–20 | |
| Terminal effector CD8+/CCR7−CD45RA+ (%) | | 8 | 8.5 | 7–32 |
| CD16+CD56+ (/μl) | | 342 | 149 | 70–480 |
| CD19+ (/μl) | | 548 | 478 | 193–628 |
| Naive B cells (CD27−IgD+) (%) | | 61.6–87.4 | ||
| Memory B cells (CD27+) (%) | | 7.6 | 7–29 | |
| Unswitched memory CD27+IgD+ (%) | | 5.7 | 2.6–13.4 | |
| Switched memory CD27+IgD− (%) | | 4–21.2 | ||
| Transitional CD24++CD38++ (%) | | 4.9 | 3.9–7.8 | |
| Plasmablast CD24−CD38++ (%) | | 0.3–1.7 | ||
| CD19+ CD21−CD38− (%) | | 1 | 0.9–3.3 | |
IgG*, under suboptimal IgG replacement therapy; MCV, mean corpuscular volume; NA, not available. Values above or below reference ranges are marked in bold.
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