Table 1.
Immunological profile of the patient.
| Patient | (normal range) | |
|---|---|---|
| Age at testing (years) | 13 | |
| Hemogram, 103 cells/ μL | ||
| White blood cells | 9.40 | (4.00 – 10.0) |
| Neutrophils | 5.92 | (2.00 – 7.50) |
| Lymphocytes | 2.35 | (1.00 – 4.00) |
| Monocytes | 0.54 | (0.20 – 1.00) |
| Lymphocyte subsets | ||
| CD3+, 103 cells/μL | 1.68 | (1.50 – 3.90) |
| CD3+CD4+, 103 cells/μL | 0.79 | (1.30 – 3.40) |
| CD45RA+CCR7+ naïve, %CD4+ | 47.5 | (31.3 – 70.0) |
| CD45RA−CCR7+central memory, % CD4+ | 23.5 | (21.0 – 41.3) |
| CD45RA−CCR7−effector memory, % CD4+ | 28.0 | (7.80 – 25.9) |
| CD45RA+CCR7−TEMRA, % CD4+ | 0.82 | (0.20 – 2.10) |
| CD3+CD8+, 103 cells/μL | 0.47 | (0.30 – 0.92) |
| CD45RA+CCR7+ naïve, % CD8+ | 67.0 | (31.1 – 73.2) |
| CD45RA−CCR7+central memory, % CD8+ | 0.76 | (2.60 – 8.70) |
| CD45RA−CCR7− effector memory, % CD8+ | 4.24 | (8.80 – 44.4) |
| CD45RA+CCR7−, TEMRA, % CD8+ | 28.0 | (8.70 – 38.0) |
| CD19+, 103 cells/μL | 0.24 | (0.21 – 0.57) |
| CD3−CD56+, 103 cells/μL | 0.31 | (0.07 – 0.48) |
| Immunoglobulins, mg/dL | ||
| IgG | 1097 | (639 – 1344) |
| IgM | 989 | (34 – 215) |
| IgA | 76 | (55 – 276) |
| Laboratory data supportive of ALPS | ||
| Hemoglobin | 9.3 | (11.0 – 13.5) |
| Platelets, 103 cells/μL | 108 | (150 – 450) |
| % CD8 CD4− cells among TCRαβ+ cells | 12.1 | (< 2.5) |
| Direct Coombs test | Positive | (negative) |
| Vitamin B12 (pg/mL) | 807 | (190 – 778) |
Bold values are outside the normal range.