Abstract: SA-PO232
IgA Heavy Chain Monoclonal Immunoglobulin Deposition Disease in a Kidney Transplant Recipient
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1802 Transplantation: Clinical
Authors
- El Sabbagh, Rana, Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Kapp, Meghan E., Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Birdwell, Kelly A., Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Concepcion, Beatrice P., Vanderbilt University Medical Center, Nashville, Tennessee, United States
Introduction
IgA heavy chain deposition disease should be differentiated from disease with polytypic IgA deposits given distinct clinical, histological and pathophysiologic features. Here, we present a case of IgA heavy chain monoclonal immunoglobulin deposition disease in a transplant recipient with a reported primary disease of IgA nephropathy.
Case Description
A 49-year-old man with a reported history of IgA nephropathy who failed medical therapy including cyclophosphamide, prednisone and mycophenolate resulting in ESRD. He received a renal allograft 3 years prior which was reportedly lost due to recurrent disease. He underwent a second living unrelated transplant with alemtuzumab/steroid induction and tacrolimus/mycophenolate/steroid maintenance immunosuppression. Two years posttransplant, he developed worsening kidney function with a SCr of 2 mg/dL (baseline 1.6 mg/dL) and new proteinuria of 3.7g/g. Kidney biopsy was performed and light microscopy revealed mild to moderate mesangial expansion with apparent eosinophilic deposits and segmental double contours of the glomerular basement membrane (GBM). Immunofluorescence, showed 3+ pseudolinear staining along GBM and tubular basement membranes (TBM), smudgy mesangial staining and vascular staining for IgA without corresponding light chain staining. By electron microscopy, punctate amorphous deposits were present along the inner aspect of GBM and outer aspect of TBM and around vascular smooth muscle cells with mesangial deposits showing focal short fibrillary substructure. He was referred to hematology and a bone marrow biopsy which showed a plasma cell neoplasm. He was started on bortezomib and dexamethasone but after 1 cycle developed severe AKI requiring HD. He did not recover function and was ultimately declared ESRD.
Discussion
IgA heavy chain monoclonal immunoglobulin deposition disease is a distinct clinico-pathologic entity which if left unrecognized or untreated can cause kidney allograft loss. This disease warrants a careful hematologic work-up to evaluate for a plasma cell dyscrasia and progression towards symptomatic IgA multiple myeloma. Although unsuccessful in this case, anti-myeloma agents appear to favorably influence renal prognosis. A hematological response may ultimately permit successful kidney transplantation with improved graft viability and decreased risk of recurrence.