Argentinean HIV-PCT patients, revision during the last 15 years.

PARERA, VICTORIA ESTELA; MELITO, VIVIANA ALICIA; ROSSETTI, MARÍA VICTORIA; BATLLE, ALCIRA

Salve Regina University, Newport-Rhode Island, USA

Congreso; 2004 GORDON RESEARCH CONFERENCE ON THE CHEMISTRHY AND BIOLOGY OF TETRAPYRROLES; 2004

Porphyrias are metabolic disorders of haem biosynthesis caused by a decreased activity of specific enzymes in this pathway. Porphyria Cutanea Tarda (PCT), the most common of all porphyrias is biochemically characterized by elevated levels of highly carboxylated porphyrins, in liver, plasma, and urine. Clinical symptoms includes hyperpigmentation, skin photosensitivity with blistering on sun exposed areas, skin fragility and hypertricosis. The disease is due to subnormal activity of Uroporphyrinogen Decarboxylase (URO-D EC 4.1.1.37). There are two forms of PCT: familial PCT (F-PCT) and sporadic PCT (S-PCT) which can be differentiated by the activity of erythrocyte URO-D. Factors such alcohol, estrogens, hepatotoxic drugs, polyhalogenated compounds, mutations in the hemochromatosis gene (HFE) and infections with hepatitis viruses (HBV and HCV) and human immunodeficiency virus (HIV), have been implicated in its pathogenesis. In HIV patients significant glutathione deficiency contributing to oxidative stress has been observed. Excess endogenous production of estrogens in HIV infection could alter porphyrin metabolism. In this study we have reviewed all Argentinean PCT patients simultaneously infected with HIV during the last 15 years. Since 1989 up to date, 91 HIV positive subjects  were diagnosed as PCT patients(aged between 20 and 50 years old). From this population only 3 were women. Among these patients 48 were alcohol and drug abusers, 16 only heavy drinkers, 10 drug abusers and 41 of them were  also coinfected with HCV. In 25 patients, an overlapping of alcohol, drug abuse and HCV infection was found, representing  27% of HIV-PCT cases. In 13 of these patients URO-D activity was measured, in only 2 cases, belonging to PCT families previously diagnosed, URO-D was diminished. In our experience treatment with low doses of chloroquine or the combined therapy with SAMe are well tolerated, with remission of cutaneous symptoms and biochemical parameters. Our conclusion is that it should be considered that HIV infected subjects are susceptible of developing Porphyria.