NKT细胞淋巴瘤周剑峰.pptVIP

发病机制 Highly activated yet ineffective multisystem inflammatory response/ Immunopathology 噬血细胞性淋巴组织增生症（HLH）发病机制 IL-1, IL-6, TNF-α etc. Tissue Infiltration（组织浸润） Cytokine Storm MSOF多系统和器官衰竭 IFN-γ 诊断 (HLH-2004) 鉴别诊断 原发性和继发性的鉴别：分子诊断 继发性HLH的病因诊断：感染（EB病毒感染最常见）、肿瘤、结缔组织病、移植、药物等 治疗 (HLH-2004) NK 细胞肿瘤：关键要点 多数起源于EBV 慢性感染后转化 疾病累及巨大的人群 诊断治疗存在盲区 具特征性改变，与B -NHL 是完全不同的疾病 基于疾病机制的新认识，诊断治疗模式正在转化 * Figure 4:?The relation between EBV infection and development of EBV-associated lymphoproliferative disease by age group.HD, Hodgkins lymphoma; ANKL, aggressive NK cell leukemia; DLBCL, diffuse large B cell lymphoma; PTCL, peripheral T cell lymphoma.*, environmental cofactor. Mentions:?In summary, EBV infection in Koreans induced predominantly T or NK cell LPDs; among these, NK/T cell lymphomas were the most common. A high prevalence of EBV infection in early childhood associated with the failure of innate immune responses to eradicate the virus resulted in the development of EBV-associated LPD throughout life. As shown in Fig. 4, primary infections in early childhood may be complicated by the development of Burkitts lymphoma and acute EBV-HLH as well as CAEBV infections; some of these transformed to ANKLs and PTCLs in young adults. In the middle-aged patients, some with chronic latent infections developed NK/T cell lymphomas and ANKL. In old patients, decreased immunity and environmental cofactors may provoke the development of PTCL and DLBCL. * 河南省肿瘤医院和中山肿瘤医院合作，今年7月发表 入组的患者已I/II期，鼻型NK/T为主 总的ORR（CR+PR）达到了91% 这么好的结果，可能与疾病分期、序贯IFRT有关 Grade 3/4 hematological toxicity was observed in only 16% of the cases (9/55). * 资料贫乏，少有研究，认识有限，分子特征和机制不明 死亡率极高，生存期短，预后极差 * * CAEBV is an EBV-associated syndrome characterized by a variety of serious hematological dis-orders, including malignant lymphoma. EBV was found to infect circulating T and/or NK cells in patients with CAEBV. These EBV-infected cells express EBNA-1, LMP-1, and LMP-2A, a type II form of EBV latency, which is also observed in nasophar