Table 1.

Hematological and immunological features of the patient

PatientReference values
Age at evaluation (years) 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 (%) ​ 1.4 2–7 
Effector memory CD8+/CCR7CD45RA (%) ​ 12 8.1 11–20 
Terminal effector CD8+/CCR7CD45RA+ (%) ​ 8.5 7–32 
NK cells 
CD16+CD56+ (/μl) ​ 342 149 70–480 
B lymphocytes 
CD19+ (/μl) ​ 548 478 193–628 
Naive B cells (CD27IgD+) (%) ​ 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 CD24CD38++ (%) ​ 0.2 0 0.3–1.7 
CD19+ CD21CD38 (%) ​ 0.8 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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