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Klinische Pathologie der renalen Amyloidosen

Clinical pathology of renal amyloidosis

  • Leitthema
  • Published:
Der Nephrologe Aims and scope

Zusammenfassung

Renale Symptome (große Proteinurie, nephrotisches Syndrom, beginnende Niereninsuffizienz) und ihre bioptische Abklärung führen am häufigsten zur Ersterkennung einer systemischen Amyloidose. Für die individuelle Therapieplanung ist die immunhistochemische Amyloiddifferenzierung unerlässlich. Als etablierte Behandlungsstrategie gilt die Ausschaltung der jeweiligen Vorläuferproteine, entweder durch eine gegen Plasmazellen gerichtete Chemotherapie mit oder ohne Stammzelltransplantation bei AL- oder durch eine gezielte antiphlogistische Behandlung bei AA-Amyloidosen. Dadurch erzielte Therapieerfolge, die Absenkung der Proteinurie und die Verbesserung der exkretorischen Funktion, sind wahrscheinlich nicht an eine Herauslösung des im Gewebe abgelagerten Amyloids gebunden.

Abstract

Renal symptoms (e.g. severe proteinuria, nephrotic syndrome, initial renal insufficiency) and the clarification by biopsy lead most commonly to the first recognition of systemic amyloidosis. For individual planning of therapy it is necessary to carry out an immunohistochemical differentiation of amyloids. The established treatment strategy involves the elimination of the corresponding precursor protein, either by chemotherapy targeted against plasma cells with or without stem cell transplantation for AL-amyloidosis or by targeted antiphlogistic treatment for AA-amyloidosis. The resulting success of therapy, reduction of proteinuria and improvement of excretory function, is probably not dependent on a release of the amyloid stored in tissue.

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Authors and Affiliations

  1. Nierenregister am Institut für Pathologie, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland

    U. Helmchen, J. Velden & U. Kneissler

  2. III. Medizinische Klinik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland

    R.A.K. Stahl

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  1. U. Helmchen
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  2. J. Velden
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  3. U. Kneissler
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  4. R.A.K. Stahl
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Correspondence to U. Helmchen.

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Helmchen, U., Velden, J., Kneissler, U. et al. Klinische Pathologie der renalen Amyloidosen. Nephrologe 3, 203–215 (2008). https://doi.org/10.1007/s11560-008-0180-9

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