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CEN Case Reports

, Volume 7, Issue 1, pp 162–168 | Cite as

TAFRO syndrome with refractory thrombocytopenia responding to tocilizumab and romiplostim: a case report

  • Shoko Noda-Narita
  • Keiichi Sumida
  • Akinari Sekine
  • Junichi Hoshino
  • Koki Mise
  • Tatsuya Suwabe
  • Noriko Hayami
  • Masayuki Yamanouchi
  • Toshiharu Ueno
  • Hiroki Mizuno
  • Masahiro Kawada
  • Rikako Hiramatsu
  • Eiko Hasegawa
  • Naoki Sawa
  • Kenmei Takaichi
  • Kenichi Ohashi
  • Takeshi Fujii
  • Yoshifumi Ubara
Case Report

Abstract

Thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly (TAFRO) syndrome is a unique clinicopathologic subtype of multicentric Castleman’s disease that has recently been identified in Japan. However, little is known about its renal histological changes and the optimal treatment for TAFRO syndrome. An 80-year-old Japanese woman was admitted to our hospital for evaluation of severe anasarca and weight gain (10 kg in a month). She had polyneuropathy, monoclonal plasma cell proliferative disorder with positive kappa M-protein, a sclerotic bone lesion, elevation of vascular endothelial growth factor (VEGF), skin changes, and extravascular volume overload, which fulfilled the diagnostic criteria for POEMS (polyneuropathy, organomegaly, endocrinopathy, and monoclonal protein, skin changes) syndrome. However, kappa-type M-protein and thrombocytopenia with positivity of platelet-associated immunoglobulin G antibody were unusual, and fitted the diagnostic criteria for TAFRO syndrome. Renal biopsy showed diffuse endocapillary proliferative glomerulonephritis with endothelial swelling and the infiltration of monocytes and neutrophils without specific immunoglobulin deposits. Her systemic symptoms were refractory to initial treatment with high-dose melphalan and glucocorticoids. Alternative therapy with an anti-interleukin-6 (IL-6) receptor antibody (tocilizumab) effectively controlled the symptoms, while a thrombopoietin receptor agonist (romiplostim) was effective for her thrombocytopenia. Results suggest that IL-6—VEGF axis and an autoimmune mechanism may be responsible for TAFRO syndrome with clinical features of POEMS and refractory thrombocytopenia, which can be successfully treated with combination of tocilizumab and romiplostim.

Keywords

Multicentric Castleman’s disease TAFRO syndrome POEMS syndrome Tocilizumab Romiplostim 

Notes

Acknowledgements

This study was funded by the Okinaka Memorial Institute for Medical Research. We wish to thank Akira Shimizu (Department of Pathology, Nihon University School of Medicine, Tokyo, Japan) and Noriaki Hashiguchi (Department of Pathology, Keio University School of Medicine, Tokyo, Japan) for valuable advice regarding the renal pathology.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no competing interests.

Research involving human participants and/or animals

This article does not contain studies with human or animal participants.

Informed consent

Written informed consent was obtained from the patient for publication of this case report.

Supplementary material

13730_2018_319_MOESM1_ESM.tiff (164 kb)
Supplementary material 1 (TIFF 163 KB)
13730_2018_319_MOESM2_ESM.tiff (1.5 mb)
Supplementary material 2 (TIFF 1521 KB)

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Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  • Shoko Noda-Narita
    • 1
  • Keiichi Sumida
    • 1
    • 5
  • Akinari Sekine
    • 5
  • Junichi Hoshino
    • 1
    • 2
    • 5
  • Koki Mise
    • 1
  • Tatsuya Suwabe
    • 1
  • Noriko Hayami
    • 1
  • Masayuki Yamanouchi
    • 1
    • 2
  • Toshiharu Ueno
    • 1
  • Hiroki Mizuno
    • 5
  • Masahiro Kawada
    • 5
  • Rikako Hiramatsu
    • 1
  • Eiko Hasegawa
    • 5
  • Naoki Sawa
    • 1
  • Kenmei Takaichi
    • 2
    • 5
  • Kenichi Ohashi
    • 3
    • 4
  • Takeshi Fujii
    • 3
  • Yoshifumi Ubara
    • 1
    • 2
    • 5
  1. 1.Nephrology CenterToranomon Hospital KajigayaTokyoJapan
  2. 2.Okinaka Memorial Institute for Medical ResearchToranomon HospitalTokyoJapan
  3. 3.Department of PathologyToranomon HospitalTokyoJapan
  4. 4.Department of PathologyYokohama City UniversityKanagawaJapan
  5. 5.Nephrology CenterToranomon HospitalTokyoJapan

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