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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
[Disulone and hepatosiderosis]
L Darrieux1, H Adamski, B Turlin
1Service de Dermatologie, CHU Pontchaillou, Cedex, France.
Annales De Dermatologie Et De Venereologie
|October 21, 2006
Summary
Long-term Disulone use, a dapsone and iron oxalate combination, may lead to hepatosiderosis. This condition involves iron overload and hemolysis, prompting consideration for serum ferritin monitoring during treatment.
Area of Science:
- Dermatology
- Hepatology
- Pharmacology
Background:
- Disulone (dapsone + iron oxalate) is a sulfone medication prescribed for various skin conditions.
- Hepatosiderosis, a condition of iron overload in the liver, is a rare but serious potential complication.
Observation:
- Two male patients developed hepatosiderosis after prolonged Disulone treatment (17 and 25 years).
- Both patients exhibited elevated serum ferritin, free iron, and hemolysis, confirmed by liver biopsy.
- Genetic hemochromatosis was excluded in both cases due to the absence of HFE gene mutations.
Findings:
- Long-term Disulone administration was associated with secondary hepatosiderosis in these cases.
- The combination of dapsone and iron oxalate in Disulone may contribute to iron overload.
- Murine models suggest a link between dapsone and hepatic iron overload via hemolysis.
Implications:
- These findings highlight a rare but significant risk of hepatosiderosis with extended Disulone use.
- Regular monitoring of serum ferritin levels may be warranted for patients on long-term Disulone therapy.
- Further research is needed to establish a definitive causal link and optimal monitoring strategies in humans.
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