Haem arginate as effective maintenance therapy for hereditary coproporphyria

Ellen H Ma, Victoria Mar, George A Varigos, Amanda Nicoll, Gayle Ross

Research output: Contribution to journalArticleResearchpeer-review

4 Citations (Scopus)

Abstract

A 35-year-old woman presented with skin fragility and photosensitivity with blisters affecting her face and hands. Other symptoms included intermittent headache, fatigue, abdominal pain and nausea. Porphyrin studies were markedly raised, with features consistent with hereditary coproporphyria (HCP). Despite strict precautions, symptoms remained significantly problematic. Regular haem arginate infusions of 3 mg/kg per day over 4 days on a monthly basis were commenced and resulted in significant improvement of the patient's symptoms and a reduction in urinary porphobilinogen. Although haem arginate infusion is known as a treatment for severe acute attacks of HCP, the effectiveness of regular infusions as maintenance therapy has not been established. This is the first report of effective symptom control correlating with normalization of biochemical markers in a patient receiving regular haem arginate infusions for the treatment of HCP.

Original languageEnglish
Pages (from-to)135-138
Number of pages4
JournalAustralasian Journal of Dermatology
Volume52
Issue number2
DOIs
Publication statusPublished - May 2011
Externally publishedYes

Keywords

  • Acute porphyria
  • Haem arginate
  • Hereditary coproporphyria

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