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Abstract: TH-PO522

Amyloid Nephropathy in the Setting of Rosai-Dorfman Disease

Session Information

  • Trainee Case Reports - I
    October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Thomas, Maria Joana Angeles, University of California Davis Medical Center, Sacramento, California, United States
  • Jen, Kuang-Yu, University of California, Davis, Sacramento, California, United States
  • Don, Burl R., University of California Medical Center, Sacramento, California, United States
  • Young, Brian Y., UC Davis Medical Center, Sacramento, California, United States
Introduction

Rosai-Dorfman disease is a non-neoplastic histiocytic disorder characterized by massive lymphadenopathy secondary to infiltration and dilation of the lymph node sinuses by large histiocytes. Massive cervical lymphadenopathy is the hallmark, but extranodal involvement has been described in 43% of cases. It can affect most organs, with about 4% of cases affecting the kidneys.

Case Description

A 64-year-old Hispanic man with diabetes mellitus and hypertension was diagnosed with Rosai-Dorfman disease 9 years ago. He was treated initially with surgical debulking, rituximab, cyclophosphamide, vincristine and prednisone. Rituximab was used intermittently since, with the last dose given 4 years ago. On current presentation, he has dysphagia and progressive cervical, hilar, mediastinal and retroperitoneal lymphadenopathy. Creatinine was elevated at 2 mg/dL from a baseline of 1.17 mg/dL. Imaging and urological studies showed no evidence of obstruction. Diagnostic work-up revealed dysmorphic RBCs, proteinuria of 14 g/day, hypocomplementemia, and positive ANA and anti-RNP70. Hepatitis panel, HIV, PR3, and MPO were negative. There was an abnormal monoclonal protein in the gamma region but kappa/lambda ratio was normal. He was empirically treated with rituximab (375mg/m2 weekly for 4 doses) and dexamethasone 40 mg daily for disease recurrence. However, the kidney function continued to deteriorate, so a kidney biopsy was performed. Widespread amyloid was noted predominantly in the interstitium, with only focal glomerular and vascular involvement. Immunofluorescence showed weak IgG without light chain restriction and corresponded to scattered subepithelial immune deposits.

Discussion

There have been few reports of amyloidosis with Rosai-Dorfman Disease, one with generalized AA amyloidosis. We postulate that cytokine production by histiocytic cells, subsequently induced synthesis of precursor proteins facilitating amyloidogenesis in this patient. Amyloid can be found anywhere in the kidney, but glomerular deposition typically predominates in most forms. This report highlights a rare association of this histiocytic disorder and renal amyloidosis, predominantly with interstitial deposition.