肝脾肿大
免疫学
造血干细胞移植
干细胞
噬血细胞性淋巴组织细胞增多症
爱泼斯坦-巴尔病毒
医学
病毒
移植
造血
生物
病理
内科学
疾病
遗传学
作者
Guisheng Hu,Kunling Shen,Jikui Deng
出处
期刊:Chinese Journal of Clinical Hepatology
日期:2018-02-28
卷期号:32 (1): 103-107
标识
DOI:10.3760/cma.j.issn.1003-9279.2018.01.022
摘要
Chronic active EBV (CAEBV) infection presents with persistent or recurring IM-like symptoms including fever, hepatosplenomegaly, lymphadenopathy, and liver dysfunction without definite underlying diseases at the diagnosis. No causative pathogenetic mechanisms have been sufficiently clarified, these patients have high EBV-DNA load in the peripheral blood and systemic clonal expansion of EBV-infected T cells, NK cells and B cells. Their prognosis is poor with life-threatening complications including hemophagocytic lymphohistiocytosis, diverse organ failure, malignant lymphomas and so on. Allogeneic hematopoietic stem cell transplantation(Allo-HSCT)is the only effective therapy at present. This review outlines the recent progress of CAEBV clinical and fundamental research at home and abroad.
Key words:
Chronic Active EBV Infection (CAEBV); EBV-associated lymphoid tissue proliferative diseases; Hematopoietic stem cell transplantation
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