TY - JOUR
T1 - A distinct subtype of epstein-barr virus-positive t/nk-cell lymphoproliferative disorder
T2 - Adult patients with chronic active epstein-barr virus infection-like features
AU - Kawamoto, Keisuke
AU - Miyoshi, Hiroaki
AU - Suzuki, Takaharu
AU - Kozai, Yasuji
AU - Kato, Koji
AU - Miyahara, Masaharu
AU - Yujiri, Toshiaki
AU - Choi, Ilseung
AU - Fujimaki, Katsumichi
AU - Muta, Tsuyoshi
AU - Kume, Masaaki
AU - Moriguchi, Sayaka
AU - Tamura, Shinobu
AU - Kato, Takeharu
AU - Tagawa, Hiroyuki
AU - Makiyama, Junya
AU - Kanisawa, Yuji
AU - Sasaki, Yuya
AU - Kurita, Daisuke
AU - Yamada, Kyohei
AU - Shimono, Joji
AU - Sone, Hirohito
AU - Takizawa, Jun
AU - Seto, Masao
AU - Kimura, Hiroshi
AU - Ohshima, Koichi
N1 - Funding Information:
The authors thank Fumiko Arakawa, Kazutaka Nakashima, Mayumi Miura, Kanoko Miyazaki, Yuki Morotomi, Chie Kuroki, and Kaoruko Nagatomo for their technical assistance. This work was partly supported by The Tsukada Medical Foundation and The Yasuda Medical Foundation.
Publisher Copyright:
© 2018 Ferrata Storti Foundation.
PY - 2018/6/3
Y1 - 2018/6/3
N2 - 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 (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 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, 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, 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.
AB - 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 (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 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, 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, 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.
UR - http://www.scopus.com/inward/record.url?scp=85048042731&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85048042731&partnerID=8YFLogxK
U2 - 10.3324/haematol.2017.174177
DO - 10.3324/haematol.2017.174177
M3 - Article
C2 - 29242302
AN - SCOPUS:85048042731
SN - 0390-6078
VL - 103
SP - 1018
EP - 1028
JO - Haematologica
JF - Haematologica
IS - 6
ER -
