YH Fang, JG Lou, H Zhao, J Chen

Abstract

Langerhans cell histiocytosis is a rare disease involving multiple systems in children. We report a case of Langerhans cell histiocytosis in a paediatric patient presenting with prolonged fever, enlargement of the cervical and abdominal lymph nodes, hepatosplenomegaly and anaemia. He was confirmed to have chronic active Epstein-Barr virus infection and Langerhans cell histiocytosis by abdominal lymph node biopsy, according to the diagnostic criteria for active Epstein-Barr virus infection and the histopathological criterion for Langerhans cell histiocytosis. The infiltration of Epstein-Barr virus in the Langerhans cell histiocytosis lesion was also detected. The diagnosis of Langerhans cell histiocytosis accompanied by chronic active Epstein-Barr virus infection is rare. The aetiology of Langerhans cell histiocytosis is obscure. However, the role of Epstein-Barr virus infection in the pathogenesis of several diseases has been postulated because higher frequencies of Epstein-Barr virus have been detected in blood or tissue samples from patients with these diseases compared with those from the unaffected individual. However, the relationship between Epstein-Barr virus infection and Langerhans cell histiocytosis remains unclear. Reports of the presence of Epstein-Barr virus in Langerhans cell histiocytosis tissues are controversial. In our case, Epstein-Barr virus infection might have participated in the development of Langerhans cell histiocytosis, according to the patient's clinical history and the infiltration of Epstein-Barr virus in the Langerhans cell histiocytosis lesion.