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Pathological findings of progressive renal involvement in a patient with TAFRO syndrome

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Abstract

TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) is thought of as an atypical type of idiopathic multicentric Castleman’s disease. Interleukin-6, vascular endothelial growth factor (VEGF), and other cytokines are considered etiological factors. A 45-year-old woman was admitted to hospital with unknown fever and abdominal pain. She had thrombocytopenia, anasarca, proteinuria/hematuria, and slight hepatosplenomegaly. Based on her clinical course and laboratory data, she was diagnosed as having TAFRO syndrome. Kidney biopsy showed a membranoproliferative glomerulonephritis (MPGN)-like lesion containing lobulations of glomeruli, endothelial cell swelling, double contours of the glomerular basement membrane, and mesangiolysis. She was treated with methylprednisolone pulse (500 mg/day) and oral prednisolone (60 mg/day) therapy. The pleural effusion and ascites disappeared, and renal function normalized. Cyclosporine was added to prevent relapse. She went home, with no relapse 8 months after hospitalization. MPGN-like lesions were found frequently in patients with TAFRO syndrome in recent reports. However, there are few reports of pathologically confirmed cases of progressive renal involvement in TAFRO syndrome. The relationship between VEGF expression in renal tissue and the pathogenesis of renal injury in TAFRO syndrome was investigated in the present case.

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Authors and Affiliations

  1. Department of Nephrology and Hypertension, Fukushima Medical University, 1, Hikarigaoka, Fukushima, Japan

    Hirotaka Saito, Kenichi Tanaka, Momoko Fujiwara, Tsuyoshi Iwasaki, Tokutaro Numata, Akira Oda, Makoto Kanno & Junichiro James Kazama

  2. Department of Basic Pathology, Fukushima Medical University, Fukushima, Japan

    Mizuko Tanaka

  3. Department of Nephrology, Ohta Nishinouchi Hospital, Koriyama, Japan

    Masaaki Eiro & Mamoru Satoh

Authors
  1. Hirotaka Saito
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  2. Kenichi Tanaka
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  3. Momoko Fujiwara
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  4. Tsuyoshi Iwasaki
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  5. Tokutaro Numata
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  6. Akira Oda
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  7. Makoto Kanno
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  8. Mizuko Tanaka
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  9. Masaaki Eiro
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  10. Mamoru Satoh
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  11. Junichiro James Kazama
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Corresponding author

Correspondence to Kenichi Tanaka.

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All authors have no conflicts of interests affecting the present study.

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

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Informed consent was obtained from the patient in this case report.

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Saito, H., Tanaka, K., Fujiwara, M. et al. Pathological findings of progressive renal involvement in a patient with TAFRO syndrome. CEN Case Rep 8, 239–245 (2019). https://doi.org/10.1007/s13730-019-00400-9

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  • DOI: https://doi.org/10.1007/s13730-019-00400-9

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