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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Zach, D; Ablasser, K; Kolesnik, E; Hoeller, V; Fruhwald, F; Prüller, F; Reiter, C; Beham-Schmid, C; Lipp, R; Rainer, PP; Zirlik, A; Wölfler, A; Verheyen, N.
Advanced isolated light chain amyloid cardiomyopathy with negative immunofixation and normal free light chain ratio.
ESC Heart Fail. 2021; 8(4):3397-3402 Doi: 10.1002/ehf2.13381 (- Case Report) [OPEN ACCESS]
Web of Science PubMed PUBMED Central FullText FullText_MUG


Führende Autor*innen der Med Uni Graz
Verheyen Nicolas Dominik
Zach David
Co-Autor*innen der Med Uni Graz
Ablasser Klemens
Beham-Schmid Christine
Fruhwald Friedrich
Höller Viktoria
Kolesnik Ewald
Lipp Rainer
Prüller Florian
Rainer Peter
Reiter Clemens
Wölfler Albert
Zirlik Andreas

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Amyloid light chain (AL) cardiomyopathy is the most malignant specific cardiomyopathy. According to international recommendations, it should be ruled out non-invasively using the serum free light chain (FLC) ratio and immunofixation electrophoresis in both serum and urine. Here, we report on a 69-year-old female patient with new-onset heart failure with mid-range ejection fraction. Cardiac imaging was highly suggestive of cardiac amyloidosis. Amyloid scintigraphy showed faint myocardial tracer uptake according to Perugini Score 1, but immunofixation was negative and the FLC ratio was normal, despite a slight increase in lambda FLCs. Endomyocardial biopsy revealed advanced myocardial lambda immunoglobulin light chain deposition. Clinically relevant extracardiac amyloid organ infiltration could not be detected. Conclusively, non-invasive testing can in rare cases fail to exclude isolated AL amyloid cardiomyopathy. We suggest that even slight increases in serum lambda or kappa FLCs should be considered abnormal in suspected cardiac amyloidosis if non-invasive testing delivers discrepant results.
Find related publications in this database (using NLM MeSH Indexing)
Aged - administration & dosage
Amyloidosis - diagnosis
Cardiomyopathies - diagnosis
Female - administration & dosage
Humans - administration & dosage
Immunoglobulin Light Chains - administration & dosage
Immunoglobulin lambda-Chains - administration & dosage
Radionuclide Imaging - administration & dosage

Find related publications in this database (Keywords)
Cardiac amyloidosis
Monoclonal gammopathy
Endomyocardial biopsy
Light chain amyloidosis
Free light chain
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