Table 1. Classification of hereditary spherocytosisa.
Carrier | Mild spherocytosis | Moderate spherocytosis | Severe spherocytosisb | |
---|---|---|---|---|
Hemoglobin (g/dl) | Normal | 11–15 | 8–12 | 6–8 |
Reticulocytes (%) | ≤3 | 3–6 | ≥6 | ≥10 |
Bilirubin (mg/dl) | 0–1 | 1–2 | ≥2 | ≥2 |
Spectrin content (% of normal) | 100 | 80–100 | 50–80 | 40–60 |
Peripheral smear | Normal | Mild spherocytosis | Spherocytosis | Spherocytosis |
Osmotic fragility fresh blood | Normal | Normal or slightly increased | Distinctly increased | Distinctly increased |
Incubated blood | Slightly increased | Distinctly increased | Distinctly increased | Distinctly increased |
From Eber SW, Armbrust R, Schroter W. Variable clinical severity of hereditary spherocytosis: relation to erythrocytic spectrin concentration, fragility, and autohemolysis. J Pediatr 1990;117:409-416.
Values in untransfused patients.