Cryoglobulinemic Glomerulonephritis Associated With Nodal and Renal Infiltration by T-Cell Lymphoma of T-Follicular Helper Phenotype: A Case Report

Am J Kidney Dis. 2018 Oct;72(4):606-611. doi: 10.1053/j.ajkd.2018.03.024. Epub 2018 May 18.

Abstract

We present a unique case of cryoglobulinemic glomerulonephritis associated with nodal and renal infiltration by T-cell lymphoma of T-follicular helper phenotype. The patient presented with transient neurologic symptoms, severe nephritic syndrome with nephrotic-range proteinuria, and acute kidney injury. He had elevated double-stranded DNA levels, low complement levels, detectable cryoglobulin, and detectable immunoglobulin M (IgM) paraprotein. The kidney biopsy showed cryoglobulinemic glomerulonephritis with a membranoproliferative pattern and diffuse interstitial infiltrates on light microscopy; IgM, C3 but weak IgG, C1q, and negative C4d staining on immunofluorescence; and deposits with organized substructures on electron microscopy. Positron emission tomography showed diffuse uptake in bilaterally enlarged kidneys and a localized group of lymph nodes. Subsequent lymph node biopsy revealed Epstein-Barr virus-negative nodal T-cell lymphoma, which was also proven in renal tissue. The association between T-cell lymphoma, autoantibodies, and cryoglobulinemia may represent a paraneoplastic phenomenon. His renal prognosis has been excellent, but overall prognosis and survival is dictated by the clinical course of T-cell lymphoma.

Keywords: Mixed cryoglobulinemic glomerulonephritis; T-cell lymphoma; autoantibody; case report; follicular helper phenotype; hypocomplementemia; kidney biopsy; lymphoid neoplasm; lymphoproliferative infiltration; membranoproliferative glomerulonephritis (MPGN); nephrotic syndrome; paraneoplastic; renal infiltration.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Biopsy, Needle
  • Bone Marrow / pathology
  • Cryoglobulinemia / complications*
  • Cryoglobulinemia / diagnosis
  • Cyclophosphamide / therapeutic use
  • Disease Progression
  • Doxorubicin / therapeutic use
  • Follow-Up Studies
  • Glomerulonephritis, Membranoproliferative / complications
  • Glomerulonephritis, Membranoproliferative / diagnostic imaging
  • Glomerulonephritis, Membranoproliferative / pathology*
  • Humans
  • Immunohistochemistry
  • Kidney Neoplasms / diagnostic imaging
  • Kidney Neoplasms / drug therapy
  • Kidney Neoplasms / pathology*
  • Lymphoma, T-Cell / complications
  • Lymphoma, T-Cell / diagnostic imaging
  • Lymphoma, T-Cell / drug therapy
  • Lymphoma, T-Cell / pathology*
  • Male
  • Phenotype
  • Positron-Emission Tomography / methods
  • Prednisone / therapeutic use
  • Risk Assessment
  • T-Lymphocytes, Helper-Inducer / pathology*
  • Time Factors
  • Treatment Outcome
  • Vincristine / therapeutic use

Substances

  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • Prednisone

Supplementary concepts

  • CHOP protocol