Case Report: Light chain amyloidosis in a patient with low-grade B-cell lymphoma.
- 1Graduate Medical Education (GME), Hospital Corporation of America (HCA) Blake Florida Hospital, Bradenton, FL, United States.
- 2NEPHROCOR, Bradenton, FL, United States.
- 3Manatee Kidney Diseases Consultants, Bradenton, FL, United States.
Abstract
Background
Light chain (AL) amyloidosis is typically associated with plasma cell dyscrasias but can rarely arise from low-grade B-cell non-Hodgkin lymphoma. Renal involvement may present with nephrotic-range proteinuria, acute kidney injury, edema, anasarca, and hypoalbuminemia. Recognizing lymphoma-associated AL amyloidosis is essential because diagnosis requires tissue confirmation, and treatment must target the underlying clonal B-cell disorder.Case
We report a rare case of severe renal AL amyloidosis in a patient with known low-grade B-cell non-Hodgkin lymphoma previously treated with rituximab. The patient presented with witnessed syncope, progressive peripheral edema, anasarca, and hypotension. Initial evaluation showed acute kidney injury with nephrotic-range proteinuria, including a urine protein-to-creatinine ratio greater than 10 g/g. Workup for autoimmune, infectious, and malignant causes of proteinuric kidney disease revealed positive hepatitis C antibody, mildly elevated serum lambda free light chains, a mildly decreased kappa-to-lambda ratio, and serum immunofixation positive for an IgM monoclonal lambda gammopathy. Renal biopsy demonstrated severe AL amyloidosis with predominant renal involvement. Bone marrow biopsy showed low-grade B-cell non-Hodgkin lymphoma involving 85% of the marrow, with CD20-positive immunophenotyping.Conclusion
This case highlights a rare presentation of renal AL amyloidosis secondary to low-grade B-cell non-Hodgkin lymphoma. Persistent nephrotic-range proteinuria in patients with B-cell lymphoma, particularly with IgM lambda monoclonal gammopathy, should prompt evaluation for amyloidosis with renal biopsy and hematologic assessment.
Related
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- Myocardial work in light-chain and transthyretin-related amyloidosis with preserved ejection fraction: a cardiovascular magnetic resonance study
- Systemic fibrinogen Aα-chain amyloidosis in 3 Japanese raccoon dogs
BibTeX
@article{matthaeus2026case,
title = {{Case Report: Light chain amyloidosis in a patient with low-grade B-cell lymphoma.}},
author = {Alex Matthaeus and David Thomas and Hakan R Toka},
journal = {Frontiers in nephrology},
year = {2026},
month = oct,
doi = {10.3389/fneph.2026.1959759},
url = {https://europepmc.org/article/MED/42903237},
note = {CC BY},
}
RIS
TY - JOUR
TI - Case Report: Light chain amyloidosis in a patient with low-grade B-cell lymphoma.
AU - Alex Matthaeus
AU - David Thomas
AU - Hakan R Toka
PY - 2026
DA - 2026/10/06
JO - Frontiers in nephrology
DO - 10.3389/fneph.2026.1959759
UR - https://europepmc.org/article/MED/42903237
AB - Background Light chain (AL) amyloidosis is typically associated with plasma cell dyscrasias but can rarely arise from low-grade B-cell non-Hodgkin lymphoma. Renal involvement may present with nephrotic-range proteinuria, acute kidney injury, edema, anasarca, and hypoalbuminemia. Recognizing lymphoma-associated AL amyloidosis is essential because diagnosis requires tissue confirmation, and treatment must target the underlying clonal B-cell disorder.Case We report a rare case of severe renal AL amyloidosis in a patient with known low-grade B-cell non-Hodgkin lymphoma previously treated with rituximab. The patient presented with witnessed syncope, progressive peripheral edema, anasarca, and hypotension. Initial evaluation showed acute kidney injury with nephrotic-range proteinuria, including a urine protein-to-creatinine ratio greater than 10 g/g. Workup for autoimmune, infectious, and malignant causes of proteinuric kidney disease revealed positive hepatitis C antibody, mildly elevated serum lambda free light chains, a mildly decreased kappa-to-lambda ratio, and serum immunofixation positive for an IgM monoclonal lambda gammopathy. Renal biopsy demonstrated severe AL amyloidosis with predominant renal involvement. Bone marrow biopsy showed low-grade B-cell non-Hodgkin lymphoma involving 85% of the marrow, with CD20-positive immunophenotyping.Conclusion This case highlights a rare presentation of renal AL amyloidosis secondary to low-grade B-cell non-Hodgkin lymphoma. Persistent nephrotic-range proteinuria in patients with B-cell lymphoma, particularly with IgM lambda monoclonal gammopathy, should prompt evaluation for amyloidosis with renal biopsy and hematologic assessment.
SN - 2813-0626
ER -