Abstract: SA-PO173
Amyloidosis Returns
Session Information
- Onco-Nephrology: Clinical
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onco-Nephrology
- 1500 Onco-Nephrology
Authors
- Sharma, Purva D., Zucker School of Medicine at Hofstra/Northwell, Great Neck, New York, United States
- Bijol, Vanesa, Northwell Health Hofstra University, Lake Success, New York, United States
- Flores Chang, Bessy Suyin, Zucker School of Medicine at Hofstra/Northwell, Kew Gardens, New York, United States
- Jhaveri, Kenar D., Northwell Health Sys, Great Neck, New York, United States
Introduction
AL Amyloidosis is the most common type of systemic amyloidosis and frequently involves the kidney. Renal outcomes in renal AL Amyloid post autologous stem cell transplant (ASCT) depend on hematologic remission, initial organ injury and depth of organ response. We describe a case of renal AL amyloid who developed worsening kidney function and nephrotic range proteinuria 3 years post ASCT and presented a challenging diagnostic and therapeutic dilemma.
Case Description
A 71 yo male presented with nephrotic syndrome with 5 gms proteinuria and had a renal biopsy revealing renal amyloidosis with lambda restriction. There was 50% interstitial fibrosis and tubular atrophy. Workup showed IgG Lambda monoclonal protein in serum and urine, normal troponin and mildly elevated NT-pro-BNP. Skeletal survey was negative. ECHO showed EF of >70% with normal wall thickness. Bone marrow (BM) showed 70% cellularity with 15-25% plasma cells cytoplasmic lambda, and CD 38 and 138 were positive. Patient received cyclophosphamide, bortezomib and dexamethasone followed by ASCT. Following transplant, he developed acute kidney injury requiring short term dialysis with creatinine stabilizing at 1.6-1.8 g/dl and a proteinuria of 1-2 gms. BM biopsy post ASCT was negative. Serum immunofixation (SIFE) was negative. 3 years later, he presented with subacute rise in proteinuria to 8 gms and a serum creatinine of 2.5-3 mg/dl. He had a second kidney biopsy that showed lambda light chain restricted AL amyloidosis, with global sclerosis or obliteration by amyloid in 42% of glomeruli, tubular atrophy (70% of cortex), diffuse interstitial fibrosis and amyloid deposition. SIFE remained negative and Kappa/Lambda ratio was 1.3. A repeat BM biopsy was negative. Minimal residual disease testing on BM was also negative.There was no evidence of cardiac or hepatic amyloid. Considering the above, this was ascertained to be progression of the original amyloid disease and no further treatment was considered.
Discussion
We present a case of AL amyloidosis treated with chemotherapy and ASCT and remained in complete hematologic remission, but three years later presented with worsening renal failure and proteinuria with repeat kidney biopsy showing extensive amyloid deposits. This case highlights the importance of distinguishing between recurrence and progression of renal amyloidosis post ASCT by detailed hematologic work up and ruling out extra-renal disease.
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