Porphyria Cutanea Tarda Medication

Updated: Oct 19, 2020
  • Author: Anthony W Linfante, Jr, MD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

Medical therapy for porphyria cutanea tarda may be used alone or in combination with phlebotomy.

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Antimalarials

Class Summary

These agents are believed to form complexes with porphyrin molecules within hepatocytes that are then discharged into the circulation and excreted by renal mechanisms. Increased urinary iron excretion has also followed their use. Reported experience in treating children with PCT with antimalarials is limited.

Chloroquine (Aralen)

Chloroquine has anti-inflammatory activity by suppressing lymphocyte transformation and may have photoprotective effects. Chloroquine binds porphyrins and enhances excretion. It is available as a 250-mg tablet and 500-mg scored tablet and is not available as a syrup. Crush the tablet and mask the bitter taste in jam, applesauce, or other soft food.

Doses for chloroquine phosphate (125-250 mg PO twice weekly) are much lower than those used for antimalarial or photoprotective indications. Larger doses can cause severe hepatotoxicity and death. Even low-dose regimens can occasionally produce hepatic toxicity, and careful monitoring is indicated. Some clinicians begin with a single, small test dose. Hepatic transaminases and urinary porphyrin output may rise transiently after institution of therapy, returning to normal levels as treatment continues. Dosing information listed in the full drug monograph for standard antimalarial or other nonporphyria indications is inappropriate for treatment of porphyria cutanea tarda.

Hydroxychloroquine (Plaquenil)

Hydroxychloroquine inhibits chemotaxis of eosinophils, inhibits locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions. Doses for hydroxychloroquine sulfate (100-200 mg [77.5-155 mg base] PO 2-3 times/wk) are much lower than those used for antimalarial or photoprotective indications. Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. Larger doses can cause severe hepatotoxicity. Even low-dose regimens can occasionally produce hepatic toxicity, and careful monitoring is indicated. Some clinicians begin with a single, small test dose. Hepatic transaminases and urinary porphyrin output may rise transiently after institution of therapy, returning to normal levels as treatment continues. Dosing information listed in the full drug monograph for standard antimalarial or other nonporphyria indications is inappropriate for treatment of porphyria cutanea tarda.

It is available as a 200-mg tablet and is not available as a syrup. Crush tablet and mask the bitter taste in jam, applesauce, or other soft food.

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Bone Marrow Stimulants

Class Summary

In patients with anemia of chronic disease in whom venesections are relatively contraindicated, stimulation of erythropoiesis can mobilize tissue iron and may even enable low-volume phlebotomies to be performed at judicious intervals.

Epoetin alfa (Epogen, Procrit)

Epoetin alfa stimulates the division and differentiation of committed erythroid progenitor cells. It induces the release of reticulocytes from bone marrow into the blood stream.

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Chelators

Class Summary

Iron chelators form complexes with tissue iron to excrete it from the body and remove the tissue iron from use.

Deferoxamine (Desferal)

In addition to being an iron chelator, deferoxamine may reduce the compensatory increase of intestinal iron absorption often seen in remission induced by the greater degree of iron reduction achieved by phlebotomies.

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