Table 1.
Laboratory findings on initial presentation and at time of sHLH diagnosis
| Laboratory test | At the diagnosis of WD (3 months old) |
At the diagnosis of HLH (4 months old) |
Normal reference |
|---|---|---|---|
| WBC | 6.9 | 4.6 | 5.5–18 × 103/µL |
| ANC | 3.0 | 3.2 | 1.0–9.5 × 103/µL |
| ALC | 2.9 | 1.6 | 2–17 × 103/µL |
| Hemoglobin | 6.7 | 6.6 | 10.1–14.4 g/dL |
| Platelets count | 323 | 39 | 150–450 × 109/L |
| Fibrinogen | NA | 1.6 | 0.9 |
| ALP | 153 | 295 | 122–469 U/L |
| ALT | 28 | 229 | 5–40 U/L |
| AST | NA | 670 | 5–40 U/L |
| GGT | NA | 222 | 5–40 U/L |
| Albumin | 34 | 30 | 35–55 mg/dL |
|
Total bilirubin Direct bilirubin |
7 NA |
19 9 |
0.2–1 mg/dL 0.08–0.25 mg/dL |
| Serum ferritin | NA | 22,683 | 10–120 ng/mL |
| LAL activity on leucocytes | < 0.02 | < 0.02 | 0.37–2.30 nmol/punch/hr |
ALC absolute lymphocyte count, ALP alkaline phosphate, ALT alanine transaminase, ANC absolute neutrophil count, AST aspartate transaminase, GGT gamma-glutamyl transpeptidase, HLH hemophagocytic lymphohistiocytosis, NA not applicable, WBC white blood cell, WD Wolman’s disease