NK/T细胞淋巴瘤的病理组织学、免疫表型及基因研究

目的：筛选高效、方便、经济的对NK／T细胞淋巴瘤更进一步分类与诊断的检测方法。方法：收集了诊断为NK／T细胞淋巴瘤的34例患石腊包埋标本，27例发生于结外，7例发生在淋巴结。用多种抗体研究其免疫表型。EB病毒EBER探针原位杂交方法检测病毒感染。PCR方法检测T细胞受体β和γ基因的克隆性重排。结果：在34例结外淋巴瘤患中，18例CD56阳性，16例TIA-1阳性。2例皮肤淋巴瘤患中所有的瘤细胞Ki-67均阳性。在：EB病毒RNA检测中，12例发生于上呼吸消化道的病例阳性，包括鼻部的9例，以及鼻外的3例。同样在2例胃肠道淋巴瘤及1例皮肤淋巴瘤患中检测到EBER。22例上呼吸消化道淋巴瘤患中有2例检测到了TCRβ或γ基因克隆性重排，所有胃肠道和皮肤淋巴瘤患均检测到了TCR基因重排。2例其他部位结外淋巴瘤及7例结内淋巴瘤患中有5例显示TCR基因的克隆性重排。结论：大多数上呼吸消化道NK／T细胞淋巴瘤起源于NK细胞，只有一小部分为T细胞来源。然而，在皮肤及胃肠道中，NK样T细胞肿瘤更多见。在淋巴结中，NK细胞淋巴瘤很少。由于NK及NK样T细胞淋巴瘤的组织学改变相似，在进行NK细胞标志物、细胞毒性颗粒蛋白检测的同时必须检测TCR基因重排才会得到准确诊断。TCR的基因重排检测仍是鉴别NK和NK样T细胞淋巴瘤的重要标准。

Study of the pathology, immunophenotype, etiology and genetic marker of NK/T-cell lymphoma

OBJECTIVE: To study the NK/T-cell lymphoma, search for a more efficacious and simpler method and establish a standard guideline for distinguishing the NK-like T-cell lymphoma from the NK-cell lymphoma. METHODS: Thirty-four NK or T-cell lymphomas from the upper aerodigestive tract (n = 22), skin (n = 2), gastrointestinal (GI) tract (n = 2), lymph nodes (n = 7), and other sites (n = 1) were studied. Immunophenotype was analyzed by immunohistochemistry. In situ hybridization with EBER 1/2 RNA probes was performed. T-cell receptor (TCR)-beta and -gamma gene rearrangement was analyzed by polymerase chain reaction (PCR). RESULTS: Eighteen cases were positive for CD(56) and 16 for TIA-1 in 34 lymphomas cases. All tumor cells in the skin cases were positive for Ki-67. Epstein-Barr virus (EBV) mRNA was detected in 12 upper aerodigestive tumors including 9 of 12 nasal and 3 extranasal tumors. EBER was also detected in 1 of 2 skin lymphomas and both of the 2 GI lymphomas. Clonal TCR-beta and -gamma gene rearrangement was detected in 2 of 22 upper aerodigestive, all of the skin and GI lymphomas, and 6 of 9 nodal and other site lymphomas. CONCLUSION: Most upper aerodigestive NK/T-cell lymphomas are genotypically NK derivation, and a few belong to T lineage. However, NK-like T-cell lymphomas more frequently seen in skin and GI tract. Nodal NK-cell lymphoma are quite rare. These two kinds of lymphomas can only be diagnosed with additional immunohistochemical markers, EBER detection by ISH, TCR gene rearrangement or NK-cell receptors (NKRs) RNA detection. Detection of TCR rearrangement remains the important standard for the diagnosis of T-cell lymphoma.