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Abstract: PO1206

Unfavorable Vintage: Dialysis-Related Amyloidosis Discovered at Transplant

Session Information

Category: Trainee Case Report

  • 701 Dialysis: Hemodialysis and Frequent Dialysis

Authors

  • Shah, Shilpi, University of Texas Health Science Center at Houston, Houston, Texas, United States
  • Edwards, Angelina, University of Texas Health Science Center at Houston, Houston, Texas, United States
Introduction

Despite its life-sustaining potential, prolonged dialysis and inadequate clearance of middle molecules can have untoward consequences. Largely underdiagnosed, dialysis-related amyloidosis (DRA) is an effect of prolonged dialysis vintage. We present a case of DRA in a patient with nearly 25 years of dialysis-dependence, diagnosed histologically at time of transplant.

Case Description

A 60-year-old man with hypertension, on dialysis since 1996, presented for renal transplant evaluation. Hematology labs showed normal serum protein electrophoresis but elevated free kappa (κ) to lambda (λ) light chain ratio of 10.9. Bone marrow biopsy was normal but patient was found to have elevated serum β2 microglobulin (β2-m) at 23.6 µg/mL. He was listed and immediately received a diseased donor transplant. Intraoperatively, biopsy of a large iliac lymph node was submitted which showed Congo Red positive staining by light microscopy for amyloid but was negative for serum amyloid A (AA) and κ light chain and λ light chain (AL). Further testing identified the amyloid protein as β2-m. Retrospective history revealed he had suffered years of joint pain, especially in his shoulders, and had bilateral carpal tunnel surgery. He was chronically dependent on hydrocodone but since transplant he no longer experienced pain. Nadir serum creatinine was 1.3 mg/dL and repeat β2-m level decreased to 4.1 µg/mL.

Discussion

Present on all cell surfaces, β2-m is freely filtered then reabsorbed and catabolized in proximal tubules. Prolonged reduction in glomerular filtration leads to accumulation and deposition of amyloid fibrils particularly in periarticular structures, leading to debilitating arthritis. Although no precise treatment for DRA exists, transplantation leads to renewed clearance and dramatic improvement in symptoms. This case reiterates the importance of remaining alert for such diseases in patients with prolonged dialysis vintage.

Congo Red Staining in Vessel Wall Demonstrates Amyloid Deposition