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Pathologie entlang der sinusoidalen Wegstrecke: sinusendotheliale und perisinusoidale Befunde

Pathology along the liver sinusoids: endothelial and perisinusoidal findings

  • Schwerpunkt: Leberpathologie
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Zusammenfassung

Sinusoide können durch Veränderungen ihrer Nachbarstrukturen oder durch systemische Einflüsse in Mitleidenschaft gezogen werden. Bei vielen nichthepatozellulär bedingten Schädigungen entlang der sinusoidalen Wegstrecke fehlen klinische Leitbefunde einer Lebererkrankung. Hier kommt der Leberbiopsie eine besondere Bedeutung zu. Die Kapillarisierung der Sinusoide ist durch Schluss der Fenestrierung, Bildung einer Basalmembran und die Expression von CD34 charakterisiert. Sie kennzeichnet aktiv fortschreitende Zirrhosen. In nichtzirrhotischem Lebergewebe weist die Kapillarisierung auf eine Störung der Leberperfusion hin. In Regeneratknoten, fokalen nodulären Hyperplasien und Leberzelladenomen lassen sich afferente kapilläre Gefäßbettabschnitte von sinusoidalen Abschnitten des Blutausstroms unterscheiden. HCC besitzen im Allgemeinen ein durchweg kapillarisiertes Gefäßbett. Das Angiosarkom und das epithelioide Hämangioendotheliom sind, solange sie sich unter Erhalt der acinären Grundstruktur ohne wesentliche Beeinträchtigung der Leberzellbalken sinusoidal ausbreiten, in der Leberbiopsie leicht zu übersehen. Toxische Endothelschäden, postsinusoidale Stase sowie sinusoidale Hyperperfusion können kongestive Läsionen der Sinusoide auslösen. Die Peliosis hepatis ist durch Ruptur des sinusoidalen Gitterfasernetzes und Defekte der endothelialen Sinuswand bedingt. Bei sinusoidaler Hämokongestion ist nach Verschlüssen abführender kleiner Lebervenen zu suchen. Perisinusoidale Fibrosierungen können durch intrasinusoidale Fremdzellinfiltrate, Speichermakrozytosen und durch Aktivierung der Ito-Zellen verursacht werden. Perisinusoidale Amyloidose kann erstes Zeichen einer zugrunde liegenden B-Zell-Neoplasie sein.

Abstract

Sinusoidal alterations unrelated to primary hepatocellular damage present without characteristic clinical findings and in these cases the liver biopsy is particularly important. Capillarization of sinusoids is characterized by closing of fenestration, formation of a basal membrane and by the expression of CD 34 and is typical for active cirrhosis. In nodular regeneratory hyperplasia, capillarization indicates a local or general disturbance of perfusion. In large regenerative nodules, focal nodular hyperplasia and liver cell adenoma CD34-positive capillaries reflect afferent parts and CD 34-negative sinusoids the efferent parts of the parenchymal vascular bed. HCC generally have a completely capillarized CD34-positive vascular bed. Hepatic angiosarcomas and epithelioid hemangioendotheliomas can be easily overseen in liver biopsies, if they spread along the sinusoids without detoriation of the acinar architecture and without significant alteration of the surrounding liver cell plates. Toxic damage of endothelial cells, post-sinusoidal stasis and sinusoidal hyperperfusion are the underlying pathogenetic principles of sinusoidal injury. Rupture and loss of the perisinusoidal reticulin fibres lead to peliosis hepatis. In these cases liver biopsy might disclose occlusion of the terminal liver veins (VOD). Perisinusoidal fibrosis can be caused by intrasinusoidal accumulation of pathologic cells, advanced intrasinusoidal macrophagocytic storage diseases and by activation of the vitamin A-storing hepatic stellate cells. Perisinusoidal amyloidosis can be the first sign of an underlying B-cell neoplasia.

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Danksagung

Für die Herstellung der Schnittpräparate und Färbungen sei Frau Susanne Steiner und Frau Barbara Reddemann, für die Hilfe bei der Bildbearbeitung Herrn Gerrit Klemm, Anna Dormagen, Anni Lange und Maike Streit gedankt.

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  1. Institut für Pathologie, Universität Bonn, Sigmund-Freud-Straße 25, 53127, Bonn, Deutschland

    H.-P. Fischer, U. Flucke & H. Zhou

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  1. H.-P. Fischer
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  2. U. Flucke
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  3. H. Zhou
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Correspondence to H.-P. Fischer.

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Fischer, HP., Flucke, U. & Zhou, H. Pathologie entlang der sinusoidalen Wegstrecke: sinusendotheliale und perisinusoidale Befunde. Pathologe 29, 37–46 (2008). https://doi.org/10.1007/s00292-007-0962-2

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