論文

査読有り 国際誌
2018年6月

A distinct subtype of Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorder: adult patients with chronic active Epstein-Barr virus infection-like features.

Haematologica
  • Keisuke Kawamoto
  • ,
  • Hiroaki Miyoshi
  • ,
  • Takaharu Suzuki
  • ,
  • Yasuji Kozai
  • ,
  • Koji Kato
  • ,
  • Masaharu Miyahara
  • ,
  • Toshiaki Yujiri
  • ,
  • Ilseung Choi
  • ,
  • Katsumichi Fujimaki
  • ,
  • Tsuyoshi Muta
  • ,
  • Masaaki Kume
  • ,
  • Sayaka Moriguchi
  • ,
  • Shinobu Tamura
  • ,
  • Takeharu Kato
  • ,
  • Hiroyuki Tagawa
  • ,
  • Junya Makiyama
  • ,
  • Yuji Kanisawa
  • ,
  • Yuya Sasaki
  • ,
  • Daisuke Kurita
  • ,
  • Kyohei Yamada
  • ,
  • Joji Shimono
  • ,
  • Hirohito Sone
  • ,
  • Jun Takizawa
  • ,
  • Masao Seto
  • ,
  • Hiroshi Kimura
  • ,
  • Koichi Ohshima

103
6
開始ページ
1018
終了ページ
1028
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.3324/haematol.2017.174177

The characteristics of adult patients with chronic active Epstein-Barr virus infection are poorly recognized, hindering early diagnosis and an improved prognosis. We studied 54 patients with adult-onset chronic active Epstein-Barr virus infection diagnosed between 2005 and 2015. Adult onset was defined as an estimated age of onset of 15 years or older. To characterize the clinical features of these adults, we compared them to those of 75 pediatric cases (estimated age of onset <15 years). We compared the prognosis of adult-onset chronic active Epstein-Barr virus infection with that of patients with nasal-type (n=37) and non-nasal-type (n=45) extranodal NK/T-cell lymphoma. The median estimated age of onset of these lymphomas was 39 years (range, 16-86 years). Compared to patients with pediatric-onset disease, those in whom the chronic active Epstein-Barr virus infection developed in adulthood had a significantly decreased incidence of fever (P=0.005), but greater frequency of skin lesions (P<0.001). Moreover, hypersensitivity to mosquito bites and the occurrence of hydroa vacciniforme were less frequent in patients with adult-onset disease (P<0.001 and P=0.0238, respectively). Thrombocytopenia, high Epstein-Barr virus nuclear antigen antibody titer, and the presence of hemophagocytic syndrome were associated with a poor prognosis (P=0.0087, P=0.0236, and P=0.0149, respectively). Allogeneic hematopoietic stem cell transplantation may improve survival (P=0.0289). Compared to pediatric-onset chronic active Epstein-Barr virus infection and extranodal NK/T-cell lymphoma, adult-onset chronic active Epstein-Barr virus infection had a poorer prognosis (P<0.001 and P=0.0484, respectively). Chronic active Epstein-Barr virus infection can develop in a wide age range, with clinical differences between adult-onset and pediatric-onset disease. Adult-onset chronic active Epstein-Barr virus infection is a disease with a poor prognosis. Further research will be needed.

リンク情報
DOI
https://doi.org/10.3324/haematol.2017.174177
PubMed
https://www.ncbi.nlm.nih.gov/pubmed/29242302
PubMed Central
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6058795

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