| Title |
TAFRO syndrome with refractory thrombocytopenia responding to tocilizumab and romiplostim: a case report
|
|---|---|
| Published in |
CEN Case Reports, February 2018
|
| DOI | 10.1007/s13730-018-0319-0 |
| Pubmed ID | |
| Authors |
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 |
| 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. |
X Demographics
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 19 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 19 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Other | 4 | 21% |
| Researcher | 3 | 16% |
| Professor > Associate Professor | 2 | 11% |
| Student > Master | 2 | 11% |
| Student > Bachelor | 1 | 5% |
| Other | 2 | 11% |
| Unknown | 5 | 26% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 7 | 37% |
| Biochemistry, Genetics and Molecular Biology | 2 | 11% |
| Social Sciences | 1 | 5% |
| Immunology and Microbiology | 1 | 5% |
| Unknown | 8 | 42% |
Attention Score in Context
This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 23 February 2018.
All research outputs
#18,589,103
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Outputs from CEN Case Reports
#138
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Outputs of similar age
#257,279
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Outputs of similar age from CEN Case Reports
#5
of 8 outputs
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