ASSOCIATION BETWEEN THALASSEMIA MAJOR AND PORPHYRIA CUTANEA TARDA TYPE I

Porphyria cutanea tarda (PCT) is a metabolic disorder due to an impaired activity of uroporphyrinogen decarboxylase, the fifth enzyme of the heme biosynthetic pathway. Three major forms of PCT can be distinguished: sporadic (acquired; type I); hereditary (type II) and familial (type III). Hepatitis C virus infection, alcohol and drugs, as well as estrogens, are proved to be the most important triggering factors for PCT. The authors describe the first case of association between Thalassemia Major and PCT type I. We report the clinical course of a patient affected by Thalassemia major and PCT. The course was complicated by hepatitis C virus infection, iron overload, anemia and estro-progestinic supplement. The authors discuss the weight that the copresence of the two different pathologies had in the choice of the therapeutic protocol. This is the first case of PCT associated with Thalassemia Major, where the use of low dosages of chloroquine resulted in improvement of clinical and biochemical picture of PCT.