期刊
CRITICAL REVIEWS IN ONCOLOGY HEMATOLOGY
卷 44, 期 3, 页码 259-272出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/S1040-8428(02)00117-8
关键词
Epstein-Barr virus; hemophagocytic lymphohistiocytosis; cytokine; multiple organ failure; treatment strategy
Epstein-Barr virus (EBV) is the major triggering factor producing hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH). In this review, diagnostic problems, clinical and histopathological features, and treatment strategies of EBV-HLH have been described. In patients with EBV-HLH, the EBV-infected T cells or natural killer (NK) cells are mostly mono- or oligoclonally proliferating, where hypercytokinemia plays a major role and causes hemophagocytosis, cellular damage and dysfunction of various organs. Although the majority of EBV-HLH cases develop in apparently immunocompetent children and adolescents, it also occurs in association with infectious mononucleosis, chronic active EBV infection, familial HLH, X-linked lymphoproliferative disease, lymphoproliferative disease like peripheral T-cell lymphoma and NK cell leukemia. In terms of treatment, special therapeutic measures are required to control the cytokine storm generated by EBV and to suppress proliferating EBV-genome-containing cells, because the clinical courses are often fulminant and result in a poor outcome. (C) 2002 Elsevier Science Ireland Ltd. All rights reserved.
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