Measurement of liver iron content by magnetic resonance imaging in 20 patients with overt porphyria cutanea tarda before phlebotomy therapy: a prospective study.
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 micromol/g) in 9 cases, slightly increased (40-59 micromol/g) in 3 cases, moderately increased (60-99 micromol/g) in 6 cases or markedly increased (100-199 micromol/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.
Dereure
Olivier
O
Department of Dermatology, University of Montpellier I, Hôpital Saint Eloi, Montpellier, France. o-dereure@chu-montpellier.fr
Jumez
Nicolas
N
Bessis
Didier
D
Gallix
Benoit
B
Guillot
Bernard
B
D000328:Adult; D005260:Female; D005293:Ferritins / Q000097:blood; D000071020:Hemochromatosis Protein; D006526:Hepatitis C / Q000150:complications; D015395:Histocompatibility Antigens Class I / Q000235:genetics; D006801:Humans; D007501:Iron / Q000032:analysis*; D008099:Liver / Q000737:chemistry*; D008279:Magnetic Resonance Imaging; D008297:Male; D008565:Membrane Proteins / Q000235:genetics; D008875:Middle Aged; D009154:Mutation; D018962:Phlebotomy; D017119:Porphyria Cutanea Tarda / Q000378:metabolism* / Q000628:therapy*; D011446:Prospective Studies
C103673:HFE protein, human; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I; D008565:Membrane Proteins; D005293:Ferritins; D007501:Iron