Table 3 Summary of immunologic features of the patients with telomere biology disorders
Patient | Reference Range (adults/children) | P1.1 | P1.2 | P2 | P3 |
|---|---|---|---|---|---|
Lymphocytes | 1300–3600/1700- 6900/1100–5900×106/L | 2030 | 2340 | 660 | 1370 |
Monocytes | 200-800 × 106/L | 620 | 560 | 290 | 460 |
Neutrophils | 1500-6700 × 106/L | 1750 | 2550 | 1110 | 6540 |
Basophils | 0-100 × 106/L | 10 | 40 | 10 | 20 |
Eosinophils | 30-440 × 106/L | 90 | 170 | 7 | 40 |
Platelets | 150,000–360 000 × 106/L | 218,000 | 264,000 | 62,000 | 233,000 |
B-cells (CD19+) | 200–2100/ 200–1600×106/L | 200 | 360 | 80 | 50 |
CD3+ | 900–4500/700–4200× 106/L | 1340 | 1670 | 560 | 490 |
CD3 + CD4+ | 500–2400/ 300–2000×106/L | 430 | 817 | 303 | 294 |
CD3 + CD8+ | 300–1600/00–1800×106/L | 770 | 800 | 230 | 220 |
NK-cells (CD3−CD16+ 56+) | 100–1000/ 90–900×106/L | 140 | 200 | 90 | 20 |
Plasmacytoid | 0.1–0.3% | 0.14 | NA | 0.10% | NA |
(lin−HLA-DR+CD123+CD11c−) | |||||
Monocytoid | 0.1–0.3% | 0.42 | NA | 0.24% | NA |
(lin−HLA-DR+CD123−CD11c+) | |||||
IgG | 6.8–15.0 g/L | 11.1 | NA | 14.3 | 1.9 |
IgA | 0.52–4.02 g/L | 2.95 | NA | 2.82 | 0.39 |
IgM | 0.47–2.84 g/L | 0.47 | NA | 1.44 | 0.5 |
IgE | 0–110 IU/L | 46 | NA | NA | < 2 |
Lymphocyte proliferative responses to mitogens | Phytohemagglutinin Concanavalin A | normal | NA | normal | abnormal |