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Haematologica 2018 Volume 103(6):1018-1028
12 13 14 15 Shinobu Tamura, Takeharu Kato, Hiroyuki Tagawa, Junya Makiyama,
Ferrata Storti Foundation
Non-Hodgkin Lymphoma
A distinct subtype of Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorder: adult patients with chronic active Epstein-Barr virus infection-like features
Keisuke Kawamoto,1,2 Hiroaki Miyoshi,1 Takaharu Suzuki,1,2 Yasuji Kozai,3
Koji Kato,4 Masaharu Miyahara,5 Toshiaki Yujiri,6 Ilseung Choi,7
Katsumichi Fujimaki,8 Tsuyoshi Muta,9 Masaaki Kume,10 Sayaka Moriguchi,11
16 1 1 1 Yuji Kanisawa, Yuya Sasaki, Daisuke Kurita, Kyohei Yamada,
Joji Shimono,1 Hirohito Sone,2 Jun Takizawa,2 Masao Seto,1 Hiroshi Kimura17* and Koichi Ohshima1*
1Department of Pathology, Kurume University School of Medicine; 2Department of Hematology, Endocrinology and Metabolism, Faculty of Medicine, Niigata University; 3Department of Hematology, Tokyo Metropolitan Tama Medical Center, Fuchu; 4Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka; 5Department of Hematology, Karatsu Red Cross Hospital; 6Third Department of Internal Medicine, Yamaguchi University School of Medicine, Ube; 7Department of Hematology, National Hospital Organization Kyushu Cancer Center, Fukuoka; 8Department of Hematology, Fujisawa City Hospital; 9Department of Hematology, Japan Community Health Care Organization Kyushu Hospital; 10Department of Hematology, Hiraka General Hospital, Yokote; 11Department of Pathology, Miyazaki University; 12Department of Hematology and Oncology, Wakayama Medical University; 13Department of Hematology, Sasebo City General Hospital; 14Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine; 15Department of Hematology, National Hospital Organization Nagasaki Medical Center, Omura; 16Department of Hematology and Oncology, Oji General Hospital, Tomakomai and 17Department of Virology, Faculty of Medicine, Nagoya University, Japan
*HK and KO share senior authorship.
ABSTRACT
The characteristics of adult patients with chronic active Epstein- Barr virus infection are poorly recognized, hindering early diag- nosis 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 (esti- mated 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 lym- phomas 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 occur- rence 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, respective- ly). 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,
Correspondence:
miyoshi_hiroaki@med.kurume-u.ac.jp
Received: June 6, 2017.
Accepted: December 6, 2017. Pre-published: December 14, 2017.
doi:10.3324/haematol.2017.174177
Check the online version for the most updated information on this article, online supplements, and information on authorship & disclosures: www.haematologica.org/content/103/6/1018
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