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Klinik und Therapie der AL-Amyloidose

Clinical presentation, diagnostics, and therapy of AL-amyloidosis

  • Leitthema
  • Published:
Der Nephrologe Aims and scope

Zusammenfassung

Die AL-Amyloidose ist eine monoklonale Plasmazellerkrankung, bei der Immunglobulinleichtketten oder Fragmente extrazellulär im Gewebe zu Amyloidfibrillen vernetzt abgelagert werden. Mit Ausnahme des Zentralnervensystems können alle Organe betroffen und somit funktionsgestört sein. Am häufigsten befallen sind die Nieren, initial oft mit nephrotischem Syndrom, später auch mit renaler Funktionseinschränkung. Viele Patienten sind aufgrund der Ablagerungen in den Gefäßen und im Herz hypotensiv. Die Behandlung zielt auf eine weitgehende Elimination der monoklonalen Plasmazellen, um ein Fortschreiten der Amyloidablagerungen zu verhindern oder wenigstens zu verlangsamen. Alter, Organfunktionen und andere Kriterien werden zur Patientenselektion für aggressive oder weniger belastende Behandlungsformen genutzt, um die therapieassoziierte Mortalität möglichst niedrig zu halten. Trotzdem ist die Prognose unbefriedigend. Deshalb ist es notwendig, neue Medikamente und experimentelle Therapieformen in klinischen Studien weiter zu entwickeln, und nach Ansätzen zu suchen, die eine Rückbildung der Ablagerungen ermöglichen könnten.

Abstract

In AL-amyloidosis, monoclonal plasma cells produce immunoglobulin light chains or fragments that form insoluble fibrillar proteins that are deposited in the tissue. With the exception of the central nervous system, all organs might be involved, with the consequence of functional loss. Kidney manifestation is most common, with initial nephrotic syndrome and, in later stages, renal insufficiency. Many patients suffer from hypotension due to vessel and cardiac amyloidosis. Treatment targets the elimination of the monoclonal plasma cells to a large extent to inhibit additional amyloid deposits. Patient age, organ function, and other criteria are used to select patients for chemotherapy of different levels of aggression in order to keep therapy-associated mortality low. Patient prognosis is poor. Therefore, new drugs and experimental therapies, including those that might reverse amyloid deposits, should be developed and tested in clinical trials.

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Authors and Affiliations

  1. Klinik für Hämatologie, Hämostaseologie, Onkologie und Stammzelltransplantation, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    D. Peest

  2. Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Hannover, Deutschland

    M. Haubitz

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  1. D. Peest
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Correspondence to D. Peest.

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Peest, D., Haubitz, M. Klinik und Therapie der AL-Amyloidose. Nephrologe 3, 291–296 (2008). https://doi.org/10.1007/s11560-008-0171-x

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  • DOI: https://doi.org/10.1007/s11560-008-0171-x

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