eJHaem
(Nov 2022)
Concurrent light chain amyloidosis and proximal tubulopathy: Insights into different aggregation behavior—A case report
Simone Feurstein,
Julian Zoller,
Constantin Schwab,
Sarah Schreiner,
Heiko Mundt,
Iris Breitkreutz,
Brigitte Schneider,
Jörg Beimler,
Martin Zeier,
Rüdiger Waldherr,
Stefan Gröschel,
Carsten Müller‐Tidow,
Stefan O. Schönland,
Ute Hegenbart
Affiliations
Simone Feurstein
Department of Internal Medicine, Section of Hematology, Oncology & Rheumatology University Hospital Heidelberg Heidelberg Germany
Julian Zoller
Department of Internal Medicine, Section of Hematology, Oncology & Rheumatology University Hospital Heidelberg Heidelberg Germany
Constantin Schwab
Institute of Pathology University Hospital Heidelberg Heidelberg Germany
Sarah Schreiner
Department of Internal Medicine, Section of Hematology, Oncology & Rheumatology University Hospital Heidelberg Heidelberg Germany
Heiko Mundt
Department of Internal Medicine, Section of Nephrology University Hospital Heidelberg Heidelberg Germany
Iris Breitkreutz
Department of Internal Medicine, Section of Hematology, Oncology & Rheumatology University Hospital Heidelberg Heidelberg Germany
Brigitte Schneider
Department of Internal Medicine, Section of Hematology, Oncology & Rheumatology University Hospital Heidelberg Heidelberg Germany
Jörg Beimler
Department of Internal Medicine, Section of Nephrology University Hospital Heidelberg Heidelberg Germany
Martin Zeier
Department of Internal Medicine, Section of Nephrology University Hospital Heidelberg Heidelberg Germany
Rüdiger Waldherr
Institute of Pathology University Hospital Heidelberg Heidelberg Germany
Stefan Gröschel
Oncology Center Worms Worms Germany
Carsten Müller‐Tidow
Department of Internal Medicine, Section of Hematology, Oncology & Rheumatology University Hospital Heidelberg Heidelberg Germany
Stefan O. Schönland
Department of Internal Medicine, Section of Hematology, Oncology & Rheumatology University Hospital Heidelberg Heidelberg Germany
Ute Hegenbart
Department of Internal Medicine, Section of Hematology, Oncology & Rheumatology University Hospital Heidelberg Heidelberg Germany
DOI
https://doi.org/10.1002/jha2.555
Journal volume & issue
Vol. 3,
no. 4
pp.
1377
– 1380
Abstract
Read online
Abstract Due to differences in the protein folding mechanisms, it is exceedingly rare for amyloid light chain (AL) amyloidosis and monoclonal gammopathy of renal significance (MGRS) to coexist. We herein report the first case of concurrent AL amyloidosis and a subclass of MGRS, light chain proximal tubulopathy (LCPT). The 53‐year‐old female was diagnosed with smoldering myeloma immunoglobulin G kappa and AL amyloidosis with deposits in fat and gastrointestinal tissue. The kidney biopsy did not show amyloid deposits but electron microscopy revealed the presence of LCPT with crystal formation in proximal tubular epithelial cells. This case illustrates the complex pathophysiology of protein deposition in monoclonal gammopathies.
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