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Pathologie der Amyloidosen

Pathology of the amyloidoses

Der Nephrologe Aims and scope

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Zusammenfassung

Amyloidosen sind eine sehr heterogene Krankheitsgruppe mit zerebralen und extrazerebralen Manifestationen. Über 26 verschiedene Proteine können Amyloid bilden. Die Immunglobulin-Leichtketten-assoziierte AL-Amyloidose ist die häufigste systemisch auftretende Form, gefolgt von der hereditären und nichthereditären ATTR-Amyloidose und der AA-Amyloidose. Etwa 5% aller Amyloidosen sind erblich. Frühzeitige Diagnose und korrekte Klassifikation stellen unverändert große Herausforderungen dar. Eine genaue Klassifikation des Amyloids und der Amyloidose ist für die Prognoseabschätzung und Therapieplanung unverzichtbar.

Abstract

Amyloidoses are a heterogeneous group of diseases with cerebral and extracerebral manifestations. More than 26 different amyloid proteins have been identified. Immunoglobulin light-chain-associated (AL) amyloidosis is the most prevalent systemic form, followed by hereditary and nonhereditary ATTR amyloidosis and AA amyloidosis. Approximately 5% of amyloidoses are inherited. Early diagnosis and correct classification is of paramount importance to help assess the prognosis and tailor the patient’s treatment.

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  1. Institut für Pathologie,Charité, Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland

    C. Röcken

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  1. C. Röcken
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Röcken, C. Pathologie der Amyloidosen. Nephrologe 3, 275–284 (2008). https://doi.org/10.1007/s11560-008-0169-4

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  • DOI: https://doi.org/10.1007/s11560-008-0169-4

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