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Peer-reviewedCC BYFrontiers in nephrology

Case Report: Light chain amyloidosis in a patient with low-grade B-cell lymphoma.

  1. 1Graduate Medical Education (GME), Hospital Corporation of America (HCA) Blake Florida Hospital, Bradenton, FL, United States.
  2. 2NEPHROCOR, Bradenton, FL, United States.
  3. 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.

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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  -