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.