Table I.
Immunologic profile of the patient.
| Variable | Age at time of testing* | |
|---|---|---|
| Hemogram 103 cells/µL (normal) | 12 months | 14 months |
| White blood cells (7.73–13.12) | 3.71 | 13.06 |
| Neutrophils (2.47–6.41) | 0.91 | 4.35 |
| Lymphocytes (2.32–5.49) | 2.36 | 6.99 |
| Monocytes (0.25–1.15) | 0.20 | 0.46 |
| Hemoglobin (10.4–12.5) | 7.9 | 10.2 |
| Hematocrit (30.5–36.4) | 23.6 | 29.8 |
| Platelets (223–461) | 89 | 474 |
| Lymphocyte subsets | ||
| CD3+, 103 cells/µL (1,900–6,200) | 804 | 3,651 |
| CD3+CD4+, 103 cells/µL (1,300–3,400) | 261 | 1,070 |
| CD45RA+CCR7+, % CD4+ (66.3–89.4) | 66.8 | 52.4 |
| CD45RA−CCR7+, % CD4+ (9.2–22.4) | 22.6 | 28.2 |
| CD45RA−CCR7−, % CD4+ (1.3–9.4) | 9.5 | 18.1 |
| CD45RA+CCR7−, % CD4+ (0.2–29) | 0.9 | 15 |
| CD3+CD8+, 103 cells/µL (620–2,000) | 505 | 2,328 |
| CD45RA+CCR7+, % CD8+ (57.8–82.9) | 55.9 | 70.4 |
| CD45RA−CCR7+, % CD8+ (1.7–8.5) | 6.5 | 1.5 |
| CD45RA−CCR7−, % CD8+ (5.1–25.1) | 9.5 | 13.2 |
| CD45RA+CCR7−, % CD8+ (6.4–20.8) | 14 | 15 |
| CD19+, 103 cells/µL (610–2,600) | 465 | 2,375 |
| CD27 − IgD+, % CD19+ (76.5–94.7) | ND | 90.9 |
| CD27+IgD+, % CD19+ (3–10.7) | ND | 4.5 |
| CD27+IgD−, % CD19+ (1.4–11.9) | ND | 2.7 |
| CD3 − CD56+, 103 cells/µL (160–1100) | 216 | 1,136 |
| Immunoglobulins (mg/dL) | ||
| IgG, (300–1500) | 884 | 1,386 |
| IgM, (25–115) | 80 | 87 |
| IgA, (16–100) | 52 | 28 |
| Proliferation (counts per minute) | ||
| Concavalin A (65,6999–239,344) | 34,503 | |
| Phytohemagglutinin (96,090–358,179) | 229,096 | |
| Anti-CD3 (62,927–217,761) | 76,757 | |
| T cell mitogen Background (204–2,104) | 264 | |
| Tetanus (8,544–102,895) | 5,530 | |
| Candida (6,231–197,940) | 25,213 | |
| T cell antigen Background (689–9,034) | 3,502 | |
*
At 12 months of age, testing was done while he was acutely ill and before starting IVIG replacement; at 14 months of age testing was done after recovery from HLH and after IVIG was initiated. Proliferation was not performed at 12 months due to lymphopenia and critically ill state. Bold values are outside the normal range.
**
Prior to intravenous immunoglobulin replacement