The patients with chronic active Epstein-Barr virus infection
(CAEBV) in childhood in Japan are described. Among 39
registered cases, 20 patients were males and 19 were females.
Unlike the X-linked lymphoproliferative syndrome, there was no
hereditary background. The incidence of hypersensitivity to
mosquito bites was high (31.3%) as a past history. Most
patients exhibited hepatomegaly (92.3%), splenomegaly (87.2%)
and fever (84.6%). The incidence of absent anti-EB virus
nuclear antigen titres was unexpectedly low (17.1%).
Lymphoreticular disorders and cardiovascular diseases were
major complications. Twenty-four (61.5%) patients died 6
months to 8 years after the onset, mainly of hepatic failure
(eight cases), cardiac failure (five cases), virus-associated
haemophagocytic syndrome (three cases) and haematological
malignancies (two cases). This study reveals the CAEBV in
Japan has several clinical features and should be informative
for the pathogenesis of EB virus.
Ishihara S, Okada S, Wakiguchi H, Kurashige T, Morishima T, Kawa-Ha