Table 1. Immunological features of PBMCs from the CD70-deficient patient.
Age-matched normal values | 5 yr/9 yr | |
---|---|---|
Leukocytes (cells × mm−3) | 4,400–15,500 | 7,000/7,200 |
Neutrophils (cells × mm−3) | 1,800–8,000 | 4,200/4,600 |
Monocytes (cells × mm−3) | 200–1,000 | 500/900 |
Lymphocytes (cells × mm−3) | 1,900–3,700 | 2,100/2,300 |
T cells | ||
CD3+ (cells × mm−3) | 1,200–2,600 | 1,617/1,863 |
CD4+ (cells × mm−3) | 650–1,500 | 672/805 |
CD8+ (cells × mm−3) | 370–1,100 | 714/644 |
CD4/CD8 ratio | 0.9–2.6 | 1.7/1.3 |
TCRα/β (%) | 26–85 | 68/65 |
TCRγ/δ (%) | 0.2–14 | 8/8 |
CD31+CD45RA+/CD4+ (recent naive thymic emigrant; %) | 43–55 | 52/56 |
CD45RO+/CD4+ (memory; %) | 13–30 | 19/22 |
CCR7+CD45RA+/CD8+ (naïve; %) | 52–68 | 32/36 |
CCR7+CD45RA−/CD8+ (central memory; %) | 2–4 | 2/2.5 |
CCR7−CD27−CD45RA−/CD8+ (effector memory; %) | 11–20 | 10/11 |
CCR7+CD27−CD45RA+/CD8+ (exhausted effector memory; EMRA; %) | 1–18 | 0.2/10 |
CD127lowCD25+/ CD4+ (regulatory; %) | 2–8 | 3/5 |
Va7+CD161+/CD3+ (MAIT; %) | 1–8 | 0.3/0.4 |
Va24+Vb11+CD161+/CD3+ (iNKT; %) | >0.02 | 0.006/0.004 |
T cell proliferation (cpm × 10−3) | ||
PHA (6.25 mg ml−1) | >50 | 58.5/132 |
OKT3 (50 ng ml−1) | >30 | 90/79.5 |
Tetanus toxoid | >10 | 15.5/49 |
NK cells | ||
CD16+CD56+ (cells × mm−3) | 100–480 | 63/92 |
CD16+CD56+ (%) | 4–17 | 3/4 |
B cells | ||
CD19+ (cells × mm−3) | 270–860 | 399/322 |
CD19+ (%) | 13–27 | 22/17 |
CD21+CD27+/CD19+ (memory; %) | >10 | 3/1 |
IgD+IgM+/CD19+CD21+CD27+ (marginal zone; %) | 31–51 | 26/NA |
IgD−IgM−/CD19+ CD21+CD27+ (switched; %) | 21–49 | 38/NA |
Immunoglobulin levels (g × L-1; 5 yr old) | ||
IgG (1/2/3) | 4/0.4/0.17 | 5/0.45/0.81 |
IgM | 0.5–1.1 | 0.51 |
IgA | 0.7–1.3 | 0.12 |
Different immunological parameters of the patient were tested from blood and PBMCs: numbers of blood cell populations, different T cell subsets, B cell subsets, and natural killers from PBMCs (tested by flow cytometry), T cell proliferation in response to different stimuli (evaluated by incorporation of [3H]thymidine), and serum (immunoglobulin subclasses). Values in bold correspond to values below the age-matched normal values. MAIT, mucosal-associated invariant T cell.