Zinc protoporphyrin, a useful parameter to address hyperferritinemia.
Zinc protoporphyrin (ZPP) is produced instead of heme as soon as iron support to erythropoiesis becomes insufficient. In iron deficiency the intra-erythrocytic ZPP concentration is increased. The aim of this study was to investigate whether ZPP is influenced by increased iron levels in hereditary hemochromatosis (HE) and is useful in the clarification of hyperferritinemia. Twenty HE patients and 160 patients with hyperferritinemic caused by anemia of chronic disorders, liver diseases, transfusional iron overload and hematologic or solid malignancies were enrolled. ZPP was measured using the Aviv front-face hematofluorometer (normal <or= 40 micromol/mol heme). In HE, ZPP was significantly lower (median, 20 micromol/mol heme; p = 0.0005) compared to our historical control group. At diagnosis, 15 (75%) HE patients had ZPP values <or=25 micromol/mol heme. After phlebotomy, ZPP remained unchanged (median, 23 micromol/mol heme), although the initially high ferritin concentration decreased to normal. ZPP values in the other hyperferritinemic groups were significantly higher compared to HE and control groups. In contrast to HE, ZPP values <or=25 micromol/mol heme were only observed in 11% of cases with non-transfusional hyperferritinemia. The diagnostic accuracy of a ZPP <or=25 micromol/mol heme to detect HE in non-transfused hyperferritinemic patients was 87%, with a sensitivity of 75% and a specificity of 89%. Showing significantly lower values in HE, ZPP seems to be a useful parameter in distinguishing HE from other hyperferritinemic disorders as those conditions are generally accompanied by an increased ZPP.
Metzgeroth
Georgia
G
III. Medizinische Universitätsklinik Mannheim, Medizinische Fakultät Mannheim, Universität Heidelberg, Mannheim, Germany.
Schultheis
Beate
B
Dorn-Beineke
Alexandra
A
Hehlmann
Rüdiger
R
Hastka
Jan
J
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000740:Anemia / Q000097:blood / Q000175:diagnosis*; D003937:Diagnosis, Differential; D004912:Erythrocytes / Q000737:chemistry / Q000378:metabolism*; D005260:Female; D005293:Ferritins / Q000097:blood* / Q000378:metabolism; D006432:Hemochromatosis / Q000097:blood / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011524:Protoporphyrins / Q000097:blood*
D011524:Protoporphyrins; C017803:zinc protoporphyrin; D005293:Ferritins