Li Ting, Zhang Bo, Ye Yuhong, Yin Hongfang
Department of Pathology, The First Hospital of Peking University, Beijing 100034, China.
Hum Pathol. 2006 Jan;37(1):54-60. doi: 10.1016/j.humpath.2005.09.020.
Nasal natural killer/T-cell lymphoma (N-NK/T-L) is prevalent in China. To further characterize this neoplasm, 36 cases of N-NK/T-L from 304 cases of malignant lymphomas in the north China area were investigated by histopathology, immunophenotyping, and genomic analysis of c-kit, in comparison with 11 cases of B-cell lymphoma (BCL) at the same region and 5 cases of nodal peripheral T-cell lymphoma (PTCL) (unspecified). Histopathologically, N-NK/T-L was characterized by coagulative necrosis, inflammatory background, and angiodestructive growth pattern. In 36 cases of N-NK/T-L, 27 cases (75.0%) were stained for CD45RO and 25 (72.2%) for CD3epsilon. Thirty cases (83.3%) were positive for T-cell intracellular antigen-1, 22 (61.1%) for granzyme B, 18 (50.0%) for CD56, and 11 (30.6%) for CD30, whereas none was positive for CD117. All 5 cases of PTCL displayed positive staining for CD45RO and T-cell intracellular antigen-1, 3 cases for CD3epsilon, but only 1 case for granzyme B. All 11 BCLs presented positive staining for CD20 and CD79a but negative for other antibodies. A significant relationship was observed between neoplastic cells pleomorphism and granzyme B expression (P < .05). Despite the fact that all cases were negative for CD117 staining, genomic sequences of c-kit 11 and exon 17 sequencing showed that 8 (26%) of 31 cases N-NK/T-L proved to contain genomic mutations, including 4 cases in exon 11 and 4 in exon 17. For the control group, only 1 (9%) of 11 BCLs and 1 (20%) of 5 cases of PTCL were detected to harbor mutations in exon 11. All mutations detected in 3 groups were missense by base substitution, and codes 571, 572, and 821 were hot spots. The results suggested that, in addition to histological features and routine immunophenotyping, granzyme B expression should be a more reliable marker in correct diagnosis of N-NK/T-L, and genetic analysis of c-kit mutation should be helpful in the diagnosis of this tumor.
鼻型自然杀伤/T细胞淋巴瘤（N-NK/T-L）在中国较为常见。为进一步明确该肿瘤的特征，对华北地区304例恶性淋巴瘤中的36例N-NK/T-L进行了组织病理学、免疫表型分析及c-kit基因分析，并与同一地区的11例B细胞淋巴瘤（BCL）及5例结外型外周T细胞淋巴瘤（PTCL，未特指）进行比较。组织病理学上，N-NK/T-L的特征为凝固性坏死、炎症背景及血管破坏性生长方式。在36例N-NK/T-L中，27例（75.0%）CD45RO染色阳性，25例（72.2%）CD3ε染色阳性。30例（83.3%）T细胞胞内抗原-1阳性，22例（61.1%）颗粒酶B阳性，18例（50.0%）CD56阳性，11例（30.6%）CD30阳性，而CD117均为阴性。5例PTCL中，所有病例CD45RO及T细胞胞内抗原-1染色均阳性，3例CD3ε阳性，但颗粒酶B仅1例阳性。11例BCL中，所有病例CD20及CD79a染色阳性，其他抗体均为阴性。肿瘤细胞多形性与颗粒酶B表达之间存在显著相关性（P<0.05）。尽管所有病例CD117染色均为阴性，但c-kit第11外显子和第17外显子测序结果显示，31例N-NK/T-L中有8例（26%）存在基因突变，其中第11外显子4例，第17外显子4例。对照组中，11例BCL仅1例（9%）、5例PTCL仅1例（20%）第11外显子检测到突变。3组中检测到的所有突变均为碱基置换错义突变，密码子571、572和821为热点。结果提示，除组织学特征和常规免疫表型分析外，颗粒酶B表达可能是正确诊断N-NK/T-L更可靠的标志物，c-kit基因突变分析有助于该肿瘤的诊断。
