Successful Treatment of Hemodialysis-Related Porphyria cutanea tarda with Deferoxamine

Abstract

End-stage renal failure and long-term hemodialysis treatment promote the development of genetically conditioned porphyria cutanea tarda (PCT). The clinical manifestation is triggered off by unknown factors coexisting with renal insufficiency and hemodialysis. Iron overload is often associated with the disease and is thought to play a key role in its pathogenesis. Iron removal by deferoxamine infusions is regarded as the treatment of choice for patients who cannot undergo repeated phlebotomy procedures and has been successfully used in patients with normal renal function. We report a case of hemodialysis-related PCT and iron overload in whom repeated venesections were contraindicated on account of severe anemia and treatment with deferoxamine led to a striking improvement of symptoms.

A Dialysis Case Presentation and Discussion Edited by Roger A. Rodby: Bullous Skin Lesions in a Patient Undergoing Chronic Hemodialysis

“…Deferoxamine, a chelating agent useful to reduce iron stores, has shown inconsistent results when used to treat PCT in patients on dialysis. In case series that have shown remission of PCT lesions with the use of deferoxamine (18,19), the time to achieve remission was lengthy (between 12 and 18 months). Deferasirox, an oral iron chelator approved by the FDA in 2005, shows some potential in the treatment of PCT, but has yet to be studied in patients with PCT and in patients on dialysis (20).…”

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“…UPG‐D is inhibited by iron overload, which is also believed to play a key role in the pathogenesis of PCT. Reducing hepatic iron content by repeated phlebotomies, 17,18 and by the use of recombinant erythropoietin 19 and a chelating agent like deferoxamine, 20 helps to lower porphrins and produce clinical remission. The use of phlebotomy may not be a reasonable option in some hemodialyzed patients with anemia of chronic disease.…”

Acute renal failure following deferoxamine overdose

“…As a consequence, in addition to renal involvement, other severe side effects (infections, cataracts, visual field defects, severe vision loss or more subtle signs of visual and auditory neurotoxicity, and slow growth) [1,3,4,5,6] have been reported. These have been demonstrated to be dose dependent [7].…”