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Kidney Week

Abstract: SA-PO0189

Concurrent AA and AL Amyloidosis in a Patient with Latent Tuberculosis: A Rare Overlap Syndrome

Session Information

Category: Onconephrology

  • 1700 Onconephrology

Authors

  • Nadayil, Jasmine, University of South Florida, Tampa, Florida, United States
  • Shah, Anish, University of South Florida, Tampa, Florida, United States
  • Amerson, John K., University of South Florida, Tampa, Florida, United States
  • Persaud, Steven H., University of South Florida, Tampa, Florida, United States
  • Bassil, Claude, University of South Florida, Tampa, Florida, United States
Introduction

AA amyloidosis is the most common type of amyloidosis, primarily driven by chronic infections in developing countries. On the other hand, AL (amyloid light-chain) amyloidosis is more prevalent in developed countries. In western countries, tuberculosis (TB) remains one of the most important secondary causes of AA amyloidosis. We present a rare case of concurrent AA amyloidosis and AL amyloidosis in a patient with latent TB.

Case Description

A 60-year-old Indian woman with known AL amyloidosis and IgG-Kappa smoldering multiple myeloma presented with significant fatigue and recent diagnoses of CKD and anemia. Her laboratory studies revealed hemoglobin 9.9 g/dl, creatinine 1.4 mg/dl, M-spike 1.74 g/dl, free light chain ratio 20.31, and positive serum immunofixation for IgG-kappa monoclonal protein. Urine protein electrophoresis was negative. Kidney biopsy demonstrated perivascular amyloid deposits with positive Congo red staining, confirmed as AA amyloid by mass spectrometry. Concurrently, fat pad biopsy with immunogold labeling identified AL kappa-restricted amyloid. Bone marrow biopsy revealed 30% clonal plasma cells in normocellular marrow, and PET imaging was negative for extramedullary disease. Given her 40-year history of living in India, latent TB was suspected and confirmed with a positive QuantiFERON-TB Gold test. Treatment was initiated with Rifampin. The decision to treat myeloma with Daratumumab, Revlimid, Bortezomib, and Dexamethasone showed improvement in serum free kappa and lamba light chains. However, she continues to have proteinuria and CKD 3b attributed to AA amyloidosis.

Discussion

Despite appropriate treatment for latent TB, we considered the possibility that the renal AA amyloidosis may be due to AL amyloidosis. However, the presence of latent TB complicates the picture as we cannot rule it out as a cause of AA amyloidosis. The co-occurrence of AL and AA amyloidosis in a patient with latent TB is particularly rare. It emphasizes the importance of a thorough infectious workup in patients with suspected secondary amyloidosis, even in the presence of an underlying plasma cell dyscrasia. Recognition of overlapping etiologies may have therapeutic and prognostic implications. Further studies are needed to guide management in such complex cases.

Digital Object Identifier (DOI)