From the Research
Hereditary spherocytosis is associated with elevated ferritin levels due to increased red blood cell destruction, and management includes monitoring ferritin levels and treating iron overload with phlebotomy or iron chelation therapy, as supported by recent studies 1, 2. The relationship between hereditary spherocytosis and serum ferritin levels is complex, with elevated ferritin levels resulting from increased red blood cell destruction and subsequent iron release.
- Key points to consider in management include:
- Monitoring ferritin levels regularly, with normal ranges being 20-250 ng/mL for females and 20-300 ng/mL for males
- Treating iron overload with phlebotomy (removing 500mL of blood every 1-2 weeks until ferritin normalizes) or iron chelation therapy with medications like deferasirox (starting at 20 mg/kg/day) or deferoxamine (20-40 mg/kg/day subcutaneously)
- Avoiding iron supplements and vitamin C with meals, as vitamin C enhances iron absorption
- Dietary modifications, including limiting red meat, organ meats, and iron-fortified foods
- Regular monitoring of complete blood counts, liver function tests, and ferritin levels every 3-6 months to prevent complications like organ damage from iron overload The underlying cell fragility syndrome may require additional management, including splenectomy in severe cases of hereditary spherocytosis, which can reduce hemolysis and the subsequent iron overload, as discussed in recent studies 3, 4.
- It is essential to prioritize the most recent and highest-quality studies, such as those published in 2024 1, 2, when making management decisions to ensure optimal patient outcomes and minimize morbidity, mortality, and impact on quality of life.