Table 1.
Immunologic characteristics of the patient with hypomorphic/atypical X-SCID.
| Parameters | Result | Normal range |
|---|---|---|
| Complete blood count | ||
| White blood cells (cells/µl) | 13800 | 4400 – 9500 |
| Lymphocytes (cells/µl) | 2898 | 1900 – 3700 |
| Neutrophils (cells/µ) | 7866 | 2600 – 6300 |
| Eosinophils (cells/µl) | 2139 | 0 – 200 |
| monocytes (cells/µl) | 897 | 300 – 900 |
| Hemoglobin (g/dl) | 12.5 | 12 – 17 |
| Platelet (×103 cells/µl) | 489 | 150 – 450 |
| Immunoglobulin levels | ||
| IgG (mg/dl) | 1755 | 680 – 1180 |
| IgG1 (mg/dl) | 1312.4 | 288 – 918 |
| IgG2 (mg/dl) | 144 | 44 – 375 |
| IgG3 (mg/dl) | 160.1 | 15.5 – 85.3 |
| IgG4 (mg/dl) | 44.6 | 0.4 – 99.2 |
| IgA (mg/dl) | 219 | 70 – 190 |
| IgM (mg/dl) | 145 | 32 – 98 |
| IgE (IU/ml) | 31120 | <45 |
| Lymphocyte subsets | ||
| CD3+ T cells/µl | 2434 | 1200 – 2600 |
| αβT cells (TCRαβ+TCRγδ−/CD3+)/T cells% | 83 | 85 – 95 |
| γδT cells (TCRαβ−TCRγδ+/CD3+)/T cells% | 17 | 7 – 20 |
| CD4+ T cells/µl | 449 | 650 – 1500 |
| CD4memoryCD45RO+ T cells/CD4+ T cells% | 65.5 | 13 – 30 |
| CD4NaiveCD45RA+CCR7+ T cells/CD4+ T cells% | 20.13 | 15.5 – 59.4 |
| CD4CMCD45RA-CCR7+ T cells/CD4+ T cells% | 19.32 | 12.2 – 26.2 |
| CD4EMCD45RA-CCR7- T cells/CD4+ T cells% | 54.31 | 10.6 – 34.2 |
| CD4TEMRACD45RA+CCR7- T cells/CD4+ T cells% | 6.24 | 4.5-43.6 |
| CD4RTECD45RA+CD31+/CD4+ T cells% | 24.5 | 19.4 – 60.9 |
| CD8+ T cells/µl | 1782 | 370 – 1100 |
| CD8memoryCD45RO+ T cells/CD8+ T cells% | 82 | 8 – 37 |
| CD8NaiveCD45RA+CCR7+ T cells/CD8+ T cells% | 1.6 | 5.5 – 39.7 |
| CD8CMCD45RA-CCR7+ T cells/CD8+ T cells% | 0.25 | 1.2 – 3.8 |
| CD8EMCD45RA-CCR7- T cells/CD8+ T cells% | 91.98 | 20.1– 44.7 |
| CD8TEMRACD45RA+CCR7- T cells/CD8+ T cells% | 6.17 | 21.5 – 61 |
| CD4+/CD8+ Ratio | 0.25 | 1.5 – 2.9 |
| DN T cells (CD4−CD8−/CD3+TCRαβ+) | 0.35 | 0.18 – 2.81 |
| Regulatory T cells (CD25+IL7R−/CD3+CD4+) | 11.52 | 4 – 14 |
| CD19+B cells/µl | 275 | 270 – 860 |
| Bnaive (CD27-IgD+)/CD19+ B cells % | 68.11 | 69.4 – 80.4 |
| Bswitched memory (CD27+IgD-)/CD19+ B cells % | 8.09 | 5.2 – 12.1 |
| Bunswitched memory (CD27+IgD+)/CD19+ B cells % | 7.13 | 7.5 – 12.4 |
| Btransitional (CD24++CD38++)/CD19+ B cells % | 4.3 | 4.5 – 9.2 |
| Bplasmablast (CD24-CD38++)/CD19+ B cells % | 2.43 | 0.7 – 3.5 |
| BCD21(-/low) (CD21lowCD38low)/CD19+ B cells % | 17.5 | 0.9 – 3.5 |
| CD3-CD16+CD56+ NK cells/µl | 188 | 100 – 480 |
Values in boldface and italics are abnormal.
CM, Central memory; EM, effector memory; TEMRA, Terminally differentiated T cells; RTE, Recent Thymic Emigrant, DN, Double negative.