Summary
In the cytoplasm of hepatocytes of liver biopsy from a 30 year old man, there were proteinaceous inclusions which by use of the peroxidaseantiperoxidase method reacted strongly with antihuman-fibrinogen IgG, but gave a negative reaction with antihuman-alpha-1-antitrypsin IgG and with antihuman-albumin IgG. As shown in the electron microscope, these protein inclusions were composed of densely packed, irregularly arranged tubules of 40 nm diameter. Clinically, the patient and members of his family showed primary hypofibrinogenemia which on the basis of the morphological findings has to be interpreted as the consequence of a disturbance in the secretion of fibrinogen.
Besides the well known alpha-1-antitrypsin deficiency, familial hypofibrinogenemia with hepatocellular fibrinogen storage appears to represent another example of a plasma deficiency due to failure of hepatic secretion.
Zusammenfassung
In der Leberbiopsie eines 30 Jahre alten Mannes waren im Cytoplasma vieler Leberzellen Proteineinschlüsse nachweisbar, die immuncytochemisch bei der Peroxidase-Antiperoxidase-Reaktion intensiv mit anti-human-Fibrinogen IgG reagierten, nicht jedoch mit anti-human-alpha-1-Antitrypsin IgG oder mit anti-human-Albumin-IgG. Die Einschlüsse zeigen im Elektronenmikroskop eine eigenartige Binnenstruktur mit dicht gelagerten bogenförmig verlaufenden Tubuli von 40 nm Durchmesser. Die bei dem Patienten und bei Familienangehörigen klinisch nachgewiesene Hypofibrino-genämie wird als Folge einer hepatischen Abgabestörung für Fibrinogen interpretiert.
Die familiäre Hypofibrinogenämie mit hepatocellulärer Fibrinogenspeicherung ist nach dem alpha-1-Antitrypsinmangel das zweite Beispiel für eine morphologisch nachweisbare Proteinspeicherung als Ursache einer peripheren plasmatischen Defizienz.
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Pfeifer, U., Ormanns, W. & Klinge, O. Hepatocellular fibrinogen storage in familial hypofibrinogenemia. Virchows Archiv B Cell Pathol 36, 247–255 (1981). https://doi.org/10.1007/BF02912070
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DOI: https://doi.org/10.1007/BF02912070