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Pathologie entlang der sinusoidalen Wegstrecke: intrasinusoidale Befunde

Pathology along the liver sinusoids: intrasinusoidal findings

  • Schwerpunkt: Leberpathologie
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Zusammenfassung

Pathologische intrasinusoidale Befunde sind meist durch extrahepatische oder systemische Erkrankungen bedingt. Unklares Fieber, Hepato- oder Hepatosplenomegalie, portaler Hypertonus oder leicht erhöhte Leberenzyme sind Grund für eine bioptische Abklärung mit wegweisendem histologischem Befund. Eine reaktive intrasinusoidale Lymphozytose, die insbesondere bei infektiöser Mononukleose in Erscheinung tritt, muss gegenüber prädominant intrasinusoidal manifesten malignen Lymphoproliferationen abgegrenzt werden. Vorstufen der Hämatopoese können auf eine chronische myeloproliferative Erkrankung oder einen myelodestruktiven Prozess hindeuten. Intrasinusoidale Karzinose oder Melanomatose können bei fehlender Massebildung der radiologischen Untersuchung entgehen und sind auch histologisch leicht zu übersehen, wenn die Tumorzellen so groß wie benachbarte Hepatozyten sind. Gleiches gilt für intrasinusoidale Speichermakrophagen. Unterformen genetisch determinierter makrophagozytärer Speichererkrankungen, z. B. des M. Gaucher, werden erst im Erwachsenenalter manifest. Eine begleitende perisinusoidale Fibrose und Immunhistochemie (CD68) helfen hierbei, die Hepatozyten-großen hellen intrasinusoidalen Makrophagen zu entdecken. Bei immunsupprimierten Patienten mit Fieber ist auf einen intrazellulären Erregerbefall insbesondere durch atypische Mykobakterien in disseminierten Makrophagen oder nichtgranulomatösen Kupffer-Zell-Nestern gezielt zu achten. Die Leishmaniose mit in Kupffer-Zellen gelegenen Amastigoten ist von reaktiver sinusoidaler Plasmozytose begleitet.

Abstract

Pathological findings in the liver sinusoids are mostly caused by extrahepatic or systemic diseases. Unclear fever, hepatosplenomegaly, portal hypertension or a mild elevation of liver enzymes are reasons for a liver biopsy leading to path-breaking diagnoses. Reactive intrasinusoidal lymphocytosis, especially with Epstein-Barr virus infections, has to be differentiated from predominantly intrasinusoidal lymphoproliferative malignancies. Intrasinusoidal megakaryocytes can be the first sign of a myeloproliferative or myelodestructive disease. Intrasinusoidal carcinosis and melanomatosis might present radiologically without tumor lesions and are easily overlooked histologically, in particular, if the critical cells have a similar size to hepatocytes. This also applies for intrasinusoidal storing macrophages. Gaucher’s disease type I, and some other subtypes of inborn storage diseases might present for the first time in adulthood by hepatomegaly and Kupffer cell hypertrophy. Accompanying perisinusoidal fibrosis and immunohistochemical staining (CD68) can help to detect the large pale intrasinusoidal macrophages. In immunocompromized patients with fever, particular attention must be paid to intracellular agents, especially atypical mycobacteria and yeasts in non-granulomatous nested or dispersed Kupffer cells. Leishmaniasis with amastigotes in macrophages is accompanied by reactive sinusoidal plasmocytosis.

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Danksagung

Für die Herstellung der Schnittpräparate und Färbungen danken wir Frau Susanne Steiner, für die Hilfe bei der Bildbearbeitung danken wir Herrn Gerrit Klemm und Frau Maike Streit.

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

    U. Flucke &  H.-P. Fischer

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

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Flucke, U., Fischer, HP. Pathologie entlang der sinusoidalen Wegstrecke: intrasinusoidale Befunde. Pathologe 29, 27–36 (2008). https://doi.org/10.1007/s00292-007-0961-3

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