Extranodal peripheral T-cell and NK-cell neoplasms.
Extranodal peripheral T-cell and NK-cell neoplasms.
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DOI：
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发表时间：
1999
影响因子：
3.5
通讯作者：
E. Jaffe;L. Krenács;S. Kumar;D. Kingma;M. Raffeld
中科院分区：
文献类型：
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作者：
E. Jaffe;L. Krenács;S. Kumar;D. Kingma;M. Raffeld
成熟t细胞淋巴瘤或外周t细胞淋巴瘤并不常见，仅占所有非霍奇金淋巴瘤的10% - 15%，其分类一直存在争议。与b细胞淋巴瘤相比，细胞学特征在确定疾病实体方面没有用处，细胞学分级在预测临床病程方面也没有用处。同样，t细胞或nk细胞衍生的许多实体没有特定的免疫表型。在确定t细胞和nk细胞肿瘤时，临床特征非常重要，有时比确切的起源细胞更重要。大多数结外t细胞/ nk细胞淋巴瘤具有细胞毒性表型。细胞毒性分子的表达可能导致肿瘤细胞和正常的旁观者细胞凋亡。结外T/NK细胞肿瘤的三大类是鼻、肠和皮下泛膜炎样。肝脾γ δ t细胞淋巴瘤是一种由功能不成熟的细胞毒性细胞引起的全身性疾病。许多结外t细胞和nk细胞肿瘤与eb病毒（EBV）有关；这种联系似乎与地点有关，并表现出一些地理差异。类似于这三种类型的肿瘤可发生在结外的不同部位。结外T/NK细胞淋巴瘤在免疫抑制的情况下发生的频率增加，特别是器官移植后。
Mature or peripheral T-cell lymphomas are uncommon, accounting for only 10% to 15% of all non-Hodgkin lymphomas, and their classification has been controversial. In contrast to B-cell lymphomas, cytologic features have not been useful in defining disease entities, and cytologic grade has not been useful in predicting the clinical course. Similarly, many entities of T-cell or NK-cell derivation do not have a specific immunophenotype. Clinical features are of major importance, sometimes more important than the precise cell of orgin, in defining T-cell and NK-cell neoplasms. Most extranodal T-cell/NK-cell lymphomas have a cytotoxic phenotpye. The expression of cytotoxic molecules may predispose to apoptosis by tumor cells and normal bystander cells. Three major categories of extranodal T/NK cell tumors are nasal, intestinal, and subcutaneous panniculitis-like. Hepatosplenic gamma delta T-cell lymphoma is a more systemic disease derived from functionally immature cytotoxic cells. Many extranodal T-cell and NK-cell neoplasms are associated with Epstein-Barr virus (EBV); the association seems site dependent and shows some geographic variation. Tumors resembling any of the 3 prototypes may occur in a variety of extranodal sites. Extranodal T/NK cell lymphomas occur with increased frequency in the setting of immune suppression, especially after organ transplantation.