Iron removal therapy in porphyria cutanea tarda: phlebotomy versus slow subcutaneous desferrioxamine infusion

Abstract

Twenty-five patients with overt clinical and biochemical findings of porphyria cutanea tarda took part in a study comparing intensive phlebotomy with slow subcutaneous desferrioxamine treatment. Fifteen male patients (Group A) had intensive venesection therapy. Ten patients (Group B) with associated diseases (minor thalassemia, cardiovascular impairment, pulmonary tuberculosis or severe liver cirrhosis) received 1.5 g of desferrioxamine by slow subcutaneous infusion using an automatic syringe pump 5 days a week. No patient complained of appreciable side effects. Serum iron, ferritin and uroporphyrins were normalized in all subjects by the end of treatment. The mean time necessary for complete recovery was 13.8 months (range 9-19) and 11.2 months (range 6-14) in Groups A and B, respectively. Liver function significantly improved during and after the treatments in both groups. We conclude that recovery from porphyria cutanea tarda can be achieved equally well using phlebotomy or desferrioxamine subcutaneous infusion. Phlebotomy is easily performed and remains the treatment of choice; slow subcutaneous desferrioxamine treatment, although expensive, is recommended when severe associated diseases contra-indicate venesection.

The role of transferrin in the mechanism of cellular iron uptake

“…Neither do we know if excess iron is obligatory to the clinical manifestations of PCT [125]. What we do know, however, is that liver iron depletion through phlebotomy improves the condition of patients with PCT [125,126].…”

Porfiria cutânea tardia

“…136 Renal transplantation may improve PCT. 137 Other possible treatments are the slow administration of subcutaneous deferoxamine (iron chelating agent), 138 cholestiramine 139 and oral thalidomide. 140 In the follow-up, the coexisting hepatic disease is supervised and, in order to prevent relapses, urinary porphyrins are measured, because porphyrinuria precedes dermatological manifestations.…”

Successful Treatment of Hemodialysis-Related Porphyria cutanea tarda with Deferoxamine

“…The administration of chloroquine removes uroporphyrins from the liver and has been successfully used [35,36]. Others have found chloroquine therapy too hazardous, hepatotoxic [15,37] and even ineffective [38]. Finally, plasma exchange has been suggested as a possible therapeutic approach in dialysis-related PCT by Disler et al [39].…”

“…As therapeutic alternative the iron chelating compound deferoxamine (DFX) has been suc cessfully used in patients suffering from PCT with nor mal renal but impaired hepatic function [15,16]. But there is still a lack of experience with hemodialysis-related PCT and DFX treatment.…”