Kawa Keisei, Okamura Takayuki, Yasui Masahiro, Sato Emiko, Inoue Masami
Department of Pediatrics, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo, Osaka Izumi City 594-1101, Japan.
Crit Rev Oncol Hematol. 2002 Dec;44(3):251-7. doi: 10.1016/s1040-8428(02)00116-6.
Epstein-Barr virus (EBV) is implicated in a variety of human diseases, some of which have fatal outcomes. Some EBV related diseases are considered to be candidates for the treatment of hematopoietic stem cell transplantation (HSCT). X-linked lymphoproliferative (XLP) syndrome is one of the representative diseases in which more than half of affected males die of infectious mononucleosis (IM) within a few weeks of primary infection, whereas the minority who survive have an increased risk of acquired hypogammaglobulinemia and lymphoma. Patients with XLP usually die by the age 40. Similarly, the majority of patients with chronic active EBV infection develop hemophagocytic syndrome, organ failure, opportunistic infection, and/or lymphoma and die within 5-10 years from onset. Recently, HSCT has provided successful outcomes in these patients. In this review, progress in the new therapeutic strategy is summarized, focusing on EBV-associated T/NK-cell lymphoproliferative disease (LPD), which is one of the heterogeneous EBV-associated disorder.
爱泼斯坦-巴尔病毒（EBV）与多种人类疾病有关，其中一些疾病会导致致命后果。一些与EBV相关的疾病被认为是造血干细胞移植（HSCT）治疗的候选对象。X连锁淋巴增殖性（XLP）综合征是一种典型疾病，超过半数的患病男性在初次感染后几周内死于传染性单核细胞增多症（IM），而存活下来的少数人患获得性低丙种球蛋白血症和淋巴瘤的风险增加。XLP患者通常在40岁前死亡。同样，大多数慢性活动性EBV感染患者会发展为噬血细胞综合征、器官衰竭、机会性感染和/或淋巴瘤，并在发病后5至10年内死亡。最近，HSCT已在这些患者中取得了成功的治疗效果。在本综述中，总结了新治疗策略的进展，重点关注EBV相关的T/NK细胞淋巴增殖性疾病（LPD），这是一种异质性EBV相关疾病。
