Table 1.
Immunologic characteristics of patients at diagnosis of CTLA4 haploinsufficiency, prior to initiation of abatacept and IVIg#.
| Patient 1 value [reference range] | Patient 2 value [reference range] | |
|---|---|---|
| Leukocytes (K cells/μL) | 6.02 [5.52-9.29] | 4.80 [4.00-11.00] |
| Neutrophils | 3.65 [3.04-6.06] | 3.9 [1.4-7.0] |
| Lymphocytes | 1.18 [1.17-2.30] | 0.99 [1.00-5.30] |
| T cells | ||
| CD3+, absolute (cells/μL) | 933 [1,000-2,600] | 815 [800-3,500] |
| CD3+/CD4+, absolute (cells/μL) | 675 [530-1,500] | 457 [400-2,100] |
| CD3+/CD8+, absolute (cells/μL) | 204 [330-1,100] | 288 [200-1,200] |
| CD4+/CD45RA+, absolute, naïve T cells (cells/μL) | n/a | 99 [no reference] |
| CD4+/CD45RO+, absolute, memory T cells (cells/μL) | n/a | 348 [no reference] |
| CD4+CD25+CD127low regulatory T cells (%) | 11.1 [5.9-10.2] | n/a |
| CD4+Foxp3+ regulatory T cells (%) | n/a | 10.48 [no reference] |
| Natural killer cells | ||
| CD3-/CD56+ or CD16+, absolute (cells/μl) | 111 [70-480] | n/a |
| CD3-/CD56+CD16+, absolute (cells/μl) | n/a | 50 [no reference] |
| B cells | ||
| CD19+, absolute (cells/μL) | 130 [110-570] | 139 [200-600] |
| CD19+CD27+IgD-, switched memory B cells (%) | 1.40 [8.30-27.80] | 2.3 [1.00-43.00] |
| CD19+CD27+IgD+, unswitched memory B cells | 4.90 [7.00-23.80] | 5.1 [2.00-28.00] |
| Immunoglobulin antibodies (mg/dL) | ||
| IgG | 637 [639-1,344] | 652 [717-1,463] |
| IgA | 31 [70-312] | 114 [51-220] |
| IgM | 69 [40-240] | 57 [59-220] |
| IgG1 | 367 [240-1118] | n/a |
| IgG2 | 64 [124-549] | n/a |
| IgG3 | 74 [21-134] | n/a |
| IgG4 | <1 [7-89] | n/a |
| Soluble IL2 receptor | 2977 [45-1105 unit/mL] | 1496 [175.30 - 858.20 pg/mL] |
| Vaccination response | ||
| Haemophilus influenza type B (Polysaccharide Ribose Phosphate; ng/mL) | <110 [>1,000] | n/a |
| Diphtheria IgG (IU/mL) | n/a | 0.26 [Positive] |
| Tetanus IgG (IU/mL) | 1.21 [0.15-7.00] | 0.53 [Positive] |
| Pneumococcal polysaccharide (mcg/mL) | Positive | Negative |
| Pneumococcus Type 1, IgG | 0.46 [>1.3] | 4.9 [≥2.3] |
| Pneumococcus Type 2, IgG | 0.94 [>1.3] | 0.5 [≥1.0] |
| Pneumococcus Type 3, IgG | 0.70 [>1.3] | 1.4 [≥1.8] |
| Pneumococcus Type 4, IgG | 1.26 [>1.3] | 1.1 [≥0.6] |
| Pneumococcus Type 5, IgG | 6.57 [>1.3] | 1.7 [≥10.7] |
| Pneumococcus Type 6B, IgG | 1.19 [>1.3] | 4.6 [≥4.7] |
| Pneumococcus Type 7F, IgG | 1.04 [>1.3] | n/a |
| Pneumococcus Type 8, IgG | 1.19 [>1.3] | 2.7 [≥2.9] |
| Pneumococcus Type 9N, IgG | 0.86 [>1.3] | n/a |
| Pneumococcus Type 9V, IgG | 0.18 [>1.3] | 3.4 [≥2.6] |
| Pneumococcus Type 10A, IgG | 6.20 [>1.3] | 2.6 [≥2.9] |
| Pneumococcus Type 11A, IgG | 0.87 [>1.3] | <0.4 [≥2.4] |
| Pneumococcus Type 12F, IgG | 2.16 [>1.3] | <0.4 [≥0.6] |
| Pneumococcus Type 14, IgG | 6.76 [>1.3] | 0.5 [≥7.0] |
| Pneumococcus Type 15B, IgG | 2.54 [>1.3] | 0.8 [≥3.3] |
| Pneumococcus Type 17, IgG | n/a | 4.2 [≥17.8] |
| Pneumococcus Type 17F, IgG | 5.64 [>1.3] | n/a |
| Pneumococcus Type 18C, IgG | 1.11 [>1.3] | n/a |
| Pneumococcus Type 19A, IgG | 15.29 [>1.3] | 1.6 [≥17.1] |
| Pneumococcus Type 19F, IgG | 6.36 [>1.3] | 2.6 [≥15.0] |
| Pneumococcus Type 20, IgG | 8.60 [>1.3] | n/a |
| Pneumococcus Type 20F, IgG | n/a | 0.7 [≥1.3] |
| Pneumococcus Type 22F, IgG | 2.28 [>1.3] | 10.0 [≥7.2] |
| Pneumococcus Type 23F, IgG | 0.66 [>1.3] | 14.4 [≥8.0] |
| Pneumococcus Type 33F, IgG | 2.06 [>1.3] | <0.4 [≥1.7] |
| Celiac autoantibody titers | ||
| Tissue transglutaminase, IgA (U/mL) | 0.4 [<6.9] | <1.0 [<8.0] |
| Deaminated gliadin peptide, IgG (EU/mL) | 4 [<19] | <1.0 [<8.0] |
| Endomysial antibody, IgA (titer) | n/a | Negative |
# Table 1 is limited to clinical laboratory evaluation.
n/a, not available.