[Hepatic reactions in erythropoietic protoporphyria (author's transl)].
The main hepatic change in erythropoietic protoporphyria is the deposition of protoporphyrin. Brown deposits of this pigment occur in bile canaliculi and ductules, discretely in hepatocytes, and secondarily in macrophages and Kupffer cells. The pigment is deposited in a crystalline form. Under the fluorescence microscope with a mercury maximum pressure burner (HO 50) at a wave length of 380--500 nm, it shows a typical red fluorescence even after paraffin embedding. Its crystalline structure results in a characteristic double refraction under the polarising microscope. Light-microscopically, hepatocellular reactions are characterised mainly by discrete alterations in the ergastoplasm. However, cell damage is indicated by diffusely distributed, hyaline single cell necrosis and by cytolytic piecemeal necrosis at the peripheries of hepatic lobules. Numerous, often disturbed mitoses produce binuclear and multinuclear hepatocytes. The obligatory secretion of protoporphyrin into the bile ducts leads to an alteration in the canalicular and ductular excretion apparatus which involves distinct ductular proliferation and accompanying fibrosis. Piecemeal necrosis is a further consequence of this process. The resulting histological picture is similar to sclerosing cholangitis with which it also has in common the slowly progressive development of hepatic cirrhosis.
D000328:Adult; D001653:Bile Ducts, Intrahepatic / Q000032:analysis; D006801:Humans; D007728:Kupffer Cells / Q000032:analysis; D008099:Liver / Q000032:analysis; D008107:Liver Diseases / Q000473:pathology*; D008264:Macrophages / Q000032:analysis; D008297:Male; D008856:Microscopy, Fluorescence; D008859:Microscopy, Polarization; D011164:Porphyrias / Q000473:pathology*; D011166:Porphyrins / Q000378:metabolism*; D011524:Protoporphyrins / Q000032:analysis / Q000378:metabolism*
D011166:Porphyrins; D011524:Protoporphyrins
Hepatische Reaktionen bei erythropoetischer Protoporphyrie.