Abstract: SA-PO1138
A Rare Case of Monoclonal Immunoglobulin Deposition Disease (MIDD) in a Transplant Recipient
Session Information
- Transplant Trainee Case Reports
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1902 Transplantation: Clinical
Authors
- Tasch, Joseph, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
- Cassol, Clarissa Araujo, The Ohio State University, Columbus, Ohio, United States
- Ayoub, Isabelle, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
- Kleman, Mark A., The Ohio State University, Columbus, Ohio, United States
- Singh, Priyamvada, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
Introduction
A 36-year-old male with ESKD presumed secondary to Type-1-diabetes received a kidney-pancreas transplant on 09/19/2017, complicated with pancreatic-vein thrombosis (day two post-op), requiring pancreas re-transplant 10/24/2018. He received induction with ATG (cumulative-dose 5 mg/kg) and maintenance with tacrolimus (trough average 8), and everolimus.
Case Description
He had multiple admissions for nausea, vomiting, and prerenal AKI. His creatinine rose from a nadir post-transplant of 1.24mg/dl to a range of 2 mg/dl. At 18 months post-transplant, he developed anasarca with creatinine rise to 2.9mg/dl. Work up revealed nephrotic syndrome with UPC 10gm/gm and serum albumin 2.7g/dl. Urine amylase (44U/L), serum amylase (16 U/L), c-peptide (2.8ng/mL), and HbA1c (5.7). A transplant-kidney biopsy showed combined antibody and T-cell-mediated rejection grade 1B (Banff 2017). EM showed finely granular powdery subendothelial and mesangial electron-dense deposits. IF showed 2+ linear IgG1 heavy-chains along the GBM. Findings were suggestive of MIDD. He was treated with steroids, and creatinine improved to 0.86mg/dL, though no improvement in pancreatic-function, leading to the resumption of insulin.
Discussion
The pancreas-kidney Donor was a 25-year old-white- healthy male with KDPI<20. Given the donor's age, donor-derived MIDD would be unlikely. The recipient SPEP, UPEP, and free-light-chain ratio were unremarkable. There was no pathological evidence of MIDD in his gastrointestinal and spleen biopsies. Whether the deposits are donor or recipient derived is unclear. We plan to closely monitor with yearly free light chains, and bone-marrow biopsy. Recurrence of MIDD is almost universal in kidney-allografts even without detectable paraprotein. The etiology for graft-failure remains elusive, though MIDD as a contributor is a theoretical possibility. Our case shows the importance of early transplant biopsy with the performance of IF and EM for all patients with suspected glomerular pathology.