Table of Contents

HK J Paediatr (New Series)
Vol 20. No. 4, 2015

HK J Paediatr (New Series) 2015;20:252-255

Case Report

Association of Langerhans Cell Histiocytosis with Chronic Active Epstein-Barr Virus Infection: Case Report and Review of the Literature
慢性人類皰疹Epstein-Barr病毒感染相關性朗格漢斯細胞組織細胞增生症:病例報導及文獻綜論

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.

兒童朗格漢斯細胞組織細胞增生症(LCH)是一種累及多系統的罕見病症。我們報導一例兒童LCH,臨床表現為長期反復發熱、頸部和腹部淋巴結腫大、肝脾腫大和貧血。根據慢性活動性Epstein-Barr(EB)病毒感染(CAEBV)及兒童LCH的診斷標準,通過淋巴結活檢,該患兒被確診為慢性活動性EB病毒感染和LCH。另外我們在患兒的朗格漢斯細胞組織細胞內發現EB病毒。LCH合併活動性EB病毒感染相當少見。目前LCH的病因尚不明確,因為在患有此等疾病病人的血液和組織內,檢測出EB病毒的機率比正常人為高,一般認為EB病毒感染可能在這些疾病中起一定作用。但由於LCH病人的組織內檢測EB病毒的研究結果報導尚有爭議,所以目前EB病毒感染和LCH的關係仍不能確定。我們根據病人的臨床表現以及在朗格漢斯細胞組織細胞內檢測到EB病毒,推測EB病毒感染可能在LCH的出現起一定作用。

Keyword : Aetiology; Fever; Lymph nodes

關鍵詞:病因、發熱、淋巴結

 
 

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