Measurement of Liver Iron Content by Magnetic Resonance Imaging in 20 Patients with Overt Porphyria Cutanea Tarda before Phlebotomy Therapy: A Prospective Study
Olivier Dereure, Nicolas Jumez, Didier Bessis, Benoit Gallix, Bernard Guillot
Liver iron content was evaluated by a magnetic resonance imaging-based method in 20 consecutive patients with either sporadic or familial porphyria cutanea tarda. Serum ferritin, hepatitis C infection and the presence of the 2 main mutations of the hemochromatosis gene were also investigated. All patients showed good clinical response to phlebotomy. Initial liver iron content was normal (< 40 μmol/g) in 9 cases, slightly increased (40–59 μmol/g) in 3 cases, moderately increased (60–99 μmol/g) in 6 cases or markedly increased (100–199 μmol/g)
in 2 cases). The ferritin level was raised (> 400 ng/ml) in 14/20 patients and there was no obvious relationship with liver iron. Increased liver iron content was observed more frequently in patients with hemochromatosis mutation and less frequent in those with hepatitis C infection. Clinical response to phlebotomies was slightly better in patients with increased liver iron content even slightly, but patients with normal liver iron content also responded well, which suggests that iron depletion is an outstanding treatment independent of liver iron content. This study shows that increased liver iron content is not a constant finding in patients with
porphyria cutanea tarda, especially in women, and that it is not a prerequisite for the efficiency of phlebotomy.