Liver iron overload and desferrioxamine treatment of porphyria cutanea tarda.
The aim of this paper is to evaluate invasive and non-invasive indices of iron store and compare the effectiveness of different ferrodepletive protocols in 150 patients with porphyria cutanea tarda (PCT). Iron removal was performed either by intensive phlebotomy (22 cases) or slow subcutaneous and high intravenous doses of desferrioxamine (18 and 5 cases, respectively), and several laboratory parameters were studied; among these, oligo-elements and urinary porphyrins (detected by HPLC) were taken into account before and after the treatments. Serum iron, transferrin saturation, ferritin (RIA) and nuclear magnetic resonance results were compared with invasive findings in order to detect the metal deposition in liver tissue (atomic absorption concentration, optic or electron-microscopic detection). Liver iron overload was observed in 95% of cases. Full normalization of the disease took place by all the treatments, even if it required slightly more time in the phlebotomy group. We may conclude that ferrodepletive treatments are highly effective in PCT and, considering the fact that siderosis and liver damage always accompany the disease, these treatments are proposed as first choice in such cases.
Rocchi
E
E
Chair of Terapia Medica Sistematica, University of Modena, Italy.
Cassanelli
M
M
Borghi
A
A
Paolillo
F
F
Pradelli
M
M
Pellizzardi
S
S
Vezzosi
A
A
Gallo
E
E
Baccarani Contri
M
M
Ventura
E
E
D001815:Bloodletting; D002851:Chromatography, High Pressure Liquid; D002908:Chronic Disease; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use*; D005293:Ferritins / Q000097:blood; D006801:Humans; D007501:Iron / Q000097:blood / Q000378:metabolism*; D008107:Liver Diseases / Q000188:drug therapy / Q000378:metabolism / Q000628:therapy*; D008854:Microscopy, Electron; D011164:Porphyrias / Q000188:drug therapy / Q000378:metabolism / Q000628:therapy*; D012871:Skin Diseases / Q000188:drug therapy / Q000378:metabolism / Q000628:therapy*
D005293:Ferritins; D007501:Iron; D003676:Deferoxamine