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Review
. 2023 Sep;64(9):842-847.
doi: 10.1007/s00108-023-01568-0. Epub 2023 Aug 4.

[Light chain amyloidosis]

[Article in German]
Ute Hegenbart  1 , Fabian Aus dem Siepen  2 , Stefan Schönland  3
Affiliations
Review

[Light chain amyloidosis]

[Article in German]
Ute Hegenbart et al. Inn Med (Heidelb). 2023 Sep.

Abstract
in English, German

Light chain amyloidosis (AL) is a rare protein deposition disease. It is caused by a clonal plasma cell or B‑cell disease in the bone marrow. With the exception of the central nervous system, all organs can be affected by amyloid deposits. Cardiac involvement is the most frequent organ manifestation that leads to significantly increased mortality when it is diagnosed at an advanced stage. The causal treatment of AL amyloidosis is reduction of amyloidogenic light chains by chemotherapy. Early diagnosis of the disease is essential to reduce early mortality, to effectively treat patients and to prevent further deterioration of organ function. New treatment approaches for AL amyloidosis are aimed at inhibiting amyloid formation or degradation of amyloid in organs.

Die Leichtketten(AL)-Amyloidose ist eine seltene Proteinablagerungserkrankung. Ursache ist eine klonale Plasmazell- oder B‑Zell-Erkrankung im Knochenmark. Mit Ausnahme des zentralen Nervensystems können alle Organe von den Amyloidablagerungen betroffen sein. Die kardiale Beteiligung ist die häufigste Organmanifestation und, falls fortgeschritten, prognostisch ungünstig. Die kausale Behandlung der AL-Amyloidose besteht in der Reduktion der amyloidogenen Leichtketten durch Chemotherapie. Eine Frühdiagnose der Erkrankung ist essenziell, um die Frühmortalität reduzieren, Patienten effektiv behandeln und eine weitere Funktionsverschlechterung der Organe verhindern zu können. Neue Ansätze zur Therapie der AL-Amyloidose zielen auf die Hemmung der Amyloidbildung bzw. auf den Amyloidabbau in Organen.

Keywords: AL amyloidosis, systemic; Amyloidosis/prognosis; Amyloidosis/staging; Amyloidosis/treatment; Monoclonal gammopathy.

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References

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