Table 1.
Laboratory data
| Presentation | Age 2 | Age 5 | Age 6 | |
|---|---|---|---|---|
| Hgb (g/dl) | 1.5 | 11.5 (transfused) | 8 (transfusion trough) | 10.1 (transfused) |
| MCV (fl) | 91.8 | 82.8 (transfused) | 82.4 (transfusion trough) | 84.7 (transfused) |
| Reticulocyte percent | 0.50% | 0.21% (transfused) | 0.12% (transfusion trough) | 0.29% (transfused) |
| WBC (per mm3) | 12,900 | 6500 | 2900 | 4600 |
| ANC (per mm3) | 6800 | 1400 | 0 | 700 |
| Platelets (per mm3) | 364,000 | 343,000 | 219,000 | 178,000 |
| IgG (mg/dl) | 665 | 727 | ||
| IgM (mg/dl) | 100 | 42 | ||
| IgA (mg/dl) | 45 | 59 | ||
| RBC antibody screen | Negative | Positive | Positive | Positive |
| Neutrophil antibody | Positive | |||
| Ferritin (ng/mL) | 762 | 2008 | 2315 | 2828 |
| R2* Estimated liver iron content (mg Fe/gram dry weight) | 10.86 | 3.67 | 8.22 | |
| Plasma ADA2 activity | 0.4 mU activity/ml |