Literature DB >> 29784613

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

Jennifer Li1, Marille Umakanathan2, Chow Heok P'ng3, Winny Varikatt3, Fiona Kwok4, Ming-Wei Lin5, Mirna Vucak-Dzumhur2.   

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. Crown
Copyright © 2018. Published by Elsevier Inc. All rights reserved.

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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

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Year:  2018        PMID: 29784613     DOI: 10.1053/j.ajkd.2018.03.024

Source DB:  PubMed          Journal:  Am J Kidney Dis        ISSN: 0272-6386            Impact factor:   8.860


  1 in total

1.  Delayed diagnosis of Angioimmunoblast T-cell lymphoma presenting with type II Cryoglobulinemia and acute kidney injury: a case report and narrative review of the literature.

Authors:  Xiang-Yang Li; Hai-Yan He; Shu-Ling Yue; Pearl Pai
Journal:  BMC Nephrol       Date:  2020-11-07       Impact factor: 2.388

  1 in total

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