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EB病毒感染相关的噬血细胞性淋巴组织细胞增生症的临床特征和治疗策略
引用本文:王学文. EB病毒感染相关的噬血细胞性淋巴组织细胞增生症的临床特征和治疗策略[J]. 医学研究生学报, 2004, 17(7): 637-640
作者姓名:王学文
作者单位:南京军区南京南京总医院血液科,江苏南京,210002
摘    要:EB病毒感染相关的噬血细胞性淋巴组织细胞增生症(EBV-HLH)好发于免疫缺陷的儿童和青少年,常呈暴发性经过,预后不良。由EBV感染的T/NK细胞大多呈单一或寡.克隆增殖。高细胞因子血症是本病发病的重要中间机制。抑制或清除含EBV基因组的T/NK细胞增殖,并积极控制细胞因子暴是本病治疗的关键。

关 键 词:EB病毒 噬血细胞性 淋巴组织细胞增生症 临床特征 治疗
文章编号:1008-8199(2004)07-0637-04
修稿时间:2003-06-12

Clinical features and therapeutic strategies of Epstein- Barr virus-associated hemophagocytic lymphohistiocytosis
Abstract:The majority cases of Epstein Barr virus associated hemophagocytic lymphohistiocytosis (EBV HLH) are apparently immunocompetent children and adolescents. The clinical courses are often fulminant and the poor outcome is usually. In patients with EBV HLH, the EBV infected T cells or natural killer (NK) cells are mostly mono or oligo clonally proliferating. Where hypercytokinemia plays a major pathogenetic middle mechanism. To reduce or eradicate proliferation of EBV infected T/NK cells, and aggressively control the cytokine storm generated by T/NK cells containing EBV genome are the special therapeutic measures for this disease.
Keywords:Epstein-Barr virus  Hemophagocytic  Lymphohistiocytosis  Clinical features  Treatment
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