論文

査読有り
2016年2月

Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease

AMERICAN JOURNAL OF HEMATOLOGY
  • Noriko Iwaki
  • ,
  • David C. Fajgenbaum
  • ,
  • Christopher S. Nabel
  • ,
  • Yuka Gion
  • ,
  • Eisei Kondo
  • ,
  • Mitsuhiro Kawano
  • ,
  • Taro Masunari
  • ,
  • Isao Yoshida
  • ,
  • Hiroshi Moro
  • ,
  • Koji Nikkuni
  • ,
  • Kazue Takai
  • ,
  • Kosei Matsue
  • ,
  • Mitsutoshi Kurosawa
  • ,
  • Masao Hagihara
  • ,
  • Akio Saito
  • ,
  • Masataka Okamoto
  • ,
  • Kenji Yokota
  • ,
  • Shinichiro Hiraiwa
  • ,
  • Naoya Nakamura
  • ,
  • Shinji Nakao
  • ,
  • Tadashi Yoshino
  • ,
  • Yasuharu Sato

91
2
開始ページ
220
終了ページ
226
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.1002/ajh.24242
出版者・発行元
WILEY-BLACKWELL

Multicentric Castleman disease (MCD) describes a heterogeneous group of disorders involving systemic inflammation, characteristic lymph node histopathology, and multi-organ dysfunction because of pathologic hypercytokinemia. Whereas Human Herpes Virus-8 (HHV-8) drives the hypercytokinemia in a cohort of immunocompromised patients, the etiology of HHV-8-negative MCD is idiopathic (iMCD). Recently, a limited series of iMCD cases in Japan sharing a constellation of clinical features, including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been described as TAFRO syndrome. Herein, we report clinicopathological findings on 25 patients (14 males and 11 females; 23 Japanese-born and two US-born), the largest TAFRO syndrome case series, including the first report of cases from the USA. The median age of onset was 50 years old (range: 23-72). The frequency of each feature was as follows: thrombocytopenia (21/25), anasarca (24/25), fever (21/25), organomegaly (25/25), and reticulin fibrosis (13/16). These patients frequently demonstrated abdominal pain, elevated serum alkaline phosphatase levels, and acute kidney failure. Surprisingly, none of the cases demonstrated marked hypergammoglobulinemia, which is frequently reported in iMCD. Lymph node biopsies revealed atrophic germinal centers with enlarged nuclei of endothelial cells and proliferation of endothelial venules in interfollicular zone. 23 of 25 cases were treated initially with corticosteroids; 12 patients responded poorly and required further therapy. Three patients died during the observation period (median: 9 months) because of disease progression or infections. TAFRO syndrome is a unique subtype of iMCD that demonstrates characteristic clinicopathological findings. Further study to clarify prognosis, pathophysiology, and appropriate treatment is needed. (C) 2015 Wiley Periodicals, Inc.

Web of Science ® 被引用回数 : 100

リンク情報
DOI
https://doi.org/10.1002/ajh.24242
Web of Science
https://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=JSTA_CEL&SrcApp=J_Gate_JST&DestLinkType=FullRecord&KeyUT=WOS:000369430800017&DestApp=WOS_CPL
URL
http://www.scopus.com/inward/record.url?eid=2-s2.0-84961382747&partnerID=MN8TOARS
URL
http://orcid.org/0000-0003-0995-9405

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