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Erschienen in: CEN Case Reports 1/2018

01.05.2018 | Case Report

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

verfasst von: 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

Erschienen in: CEN Case Reports | Ausgabe 1/2018

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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.
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Metadaten
Titel
TAFRO syndrome with refractory thrombocytopenia responding to tocilizumab and romiplostim: a case report
verfasst von
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
Publikationsdatum
01.05.2018
Verlag
Springer Japan
Erschienen in
CEN Case Reports / Ausgabe 1/2018
Elektronische ISSN: 2192-4449
DOI
https://doi.org/10.1007/s13730-018-0319-0

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