CHRONIC ACTIVE EPSTEIN-BARR-VIRUS INFECTION IN CHILDREN IN JAPAN

The patients with chronic active Epstein-Barr virus infection (CAEBV) in childhood in Japan are described. Among 39 registered cases, 20 pat ients were males and 19 were females. Unlike the X-linked lymphoprolif erative 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%) a nd fever (84.6%). The incidence of absent anti-EB virus nuclear antige n titres was unexpectedly low (17.1%). Lymphoreticular disorders and c ardiovascular diseases were major complications, Twenty-four (61.5%) p atients died 6 months to 8 years after the onset, mainly of hepatic fa ilure (eight cases), cardiac failure (five cases), virus-associated ha emophagocytic syndrome (three cases) and haematological malignancies ( two cases). This study reveals that CAEBV in Japan has several clinica l features and should be informative for the pathogenesis of EB virus.