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CEN Case Reports

, Volume 8, Issue 1, pp 48–54 | Cite as

An autopsy case of TAFRO syndrome with membranoproliferative glomerulonephritis-like lesions

  • Keiko Hashimoto
  • Takashi SanoEmail author
  • Yukari Honma
  • Maoko Ida
  • Hiroshi Tominaga
  • Aya Sawada
  • Tetsuya Abe
  • Haruka Takahashi
  • Yoshitaka Shimada
  • Takanori Masaki
  • Mariko Kamata
  • Shokichi Naito
  • Togo Aoyama
  • Yasuo Takeuchi
  • Masashi Akiya
  • Madoka Inukai
  • Norihiro Nakata
Case Report
  • 63 Downloads

Abstract

TAFRO syndrome (thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly) is an atypical manifestation of multicentric Castleman’s disease. Although overproduction of interleukin-6, vascular endothelial growth factor, and other cytokines may partially explain the pathophysiology of this rare syndrome, the precise mechanisms underlying the renal dysfunction associated with the condition remain unclear. Here, we describe a case of a 69-year-old male with TAFRO syndrome. He was treated with immunosuppressive agents and his renal function improved. Tapering of immunosuppressive agents resulted in a deterioration of renal function and an elevation of C-reactive protein. After 20 months of treatment, the patient died from tuberculous peritonitis and gastrointestinal bleeding. An autopsy revealed miliary tuberculosis, mediastinal lymphadenopathy, and gastric ulcers. Renal histopathology showed a membranoproliferative glomerulonephritis-like appearance. Almost all glomeruli showed lobular formations with mesangial proliferation and duplication of glomerular capillary walls on light microscopy. Immunofluorescence showed deposition of C1q and IgM along the glomerular capillary walls. Electron microscopy showed mesangial expansion and widening of the subendothelial space with a large number of electron-dense deposits. The glomerular lesions might be characteristic of TAFRO syndrome, and were regarded as the main cause of the patient’s renal dysfunction.

Keywords

TAFRO syndrome Renal dysfunction Hypercytokinemia Membranoproliferative glomerulonephritis-like lesions 

Notes

Compliance with ethical standards

Conflict of interest

The authors have declared no conflicts of interest.

Research involving human participants and/or animals

This article does not contain any studies with human participants performed by any of the authors.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  • Keiko Hashimoto
    • 1
  • Takashi Sano
    • 1
    Email author
  • Yukari Honma
    • 1
  • Maoko Ida
    • 1
  • Hiroshi Tominaga
    • 1
  • Aya Sawada
    • 1
  • Tetsuya Abe
    • 1
  • Haruka Takahashi
    • 1
  • Yoshitaka Shimada
    • 1
  • Takanori Masaki
    • 1
  • Mariko Kamata
    • 1
  • Shokichi Naito
    • 1
  • Togo Aoyama
    • 1
  • Yasuo Takeuchi
    • 1
  • Masashi Akiya
    • 2
  • Madoka Inukai
    • 2
  • Norihiro Nakata
    • 2
  1. 1.Department of NephrologyKitasato University School of MedicineSagamiharaJapan
  2. 2.Department of PathologyKitasato University School of MedicineSagamiharaJapan

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