摘要目的 观察广东地区不同类型成熟T和自然杀伤(NK)细胞/T细胞淋巴瘤及其亚型的临床病理特点.方法 按WHO(2008版)标准重新评估广东地区2002-2006年1137例成熟T和NK/T细胞淋巴瘤患者.由多名血液病理医师复查,补做必要的免疫组织化学染色及原位杂交.结果 963例确诊为成熟T和NK/T细胞淋巴瘤,占同期所有淋巴瘤20.1%(963/4801),发生于结内319例(33.1%),结外644例(66.9%);非特殊型外周T细胞淋巴瘤293例(30.4%);结外鼻型NK/T细胞淋巴瘤281例(29.2%);间变性大细胞淋巴瘤(ALCL)198例(20.6%);血管免疫母细胞性T细胞淋巴瘤(AILT)46例(4.8%).男女比为1.99:1,发病中位年龄为44岁.非特殊型外周T细胞淋巴瘤好发于55～64岁;结外鼻型NK/T细胞淋巴瘤好发于25～54岁;间变性淋巴瘤激酶(ALK)阳性ALCL多见于年轻人而阴性多见于中老年人;AIIJT好发于65～74岁.结论 广东地区成熟T和 NK/T细胞淋巴瘤多见于结外,好发于男性,总体发病与年龄增长无明显关系,但具体类型有不同的年龄侧重群;常见的类型依次为非特殊型外周T细胞淋巴瘤、结外鼻型NK/T细胞淋巴瘤及ALCL;EB病毒感染与NK/T细胞淋巴瘤关系密切.

abstractsObjective To study the clinicopathologic features of various types of mature T-cell and natural killer ( NK)/T-cell lymphoma in Guangdong, China, with respect to the 2008 WHO classification of lymphoid neoplasms. Methods Eleven hundred and thirty-seven (1137) cases of mature T-cell or NK/T-cell lymphoma diagnosed during the period from 2002 to 2006 in Guangzhou area were retrieved. The clinical data, histologic features and immunohistochemical findings were reviewed by a panel of experienced hematopathologists. Additional immunostaining was performed if indicated. The cases were re-classified according to the 2008 WHO classification of lymphoid neoplasms. Results Nine hundred and sixty-three (963) cases fulfilled the diagnostic criteria of mature T-cell or NK/T-cell lymphoma and accounted for 20. 1% of all cases of lymphoma encountered during the same period (963/4801). A predominance of extranodal involvement was noted in 644 cases (66. 9% ) , while 319 cases (33. 1% ) showed mainly nodal disease. The prevalence of various lymphoma subtypes was as follows; peripheral T-cell lymphoma, unspecified (PTCL,NOS) 293 cases (30.4%), extranodal NK/T-cell lymphoma, nasal type 281 cases (29. 2% ) , anaplastic large cell lymphoma (ALCL) 198 cases (20. 6% ) , and angioimmunoblastic T-celllymphoma (AILT) 46 cases (4. 8% ). The male-to-female ratio was 1. 99. The median age of the patients was 44 years, with the peak age of PTCL,NOS, extranodal NK/T-cell lymphoma,nasal type and AILT being 55 to 64 years, 25 to 54 years and 65 to 74 years, respectively. ALK-positive ALCL occurred more frequently in young age, while the ALK-negative ALCL cases occurred mainly in the elderly. Conclusions Extranodal lesions predominate in mature T-cell and NK/T-cell various subtypes however varies. The most common subtype was PTCL.NOS, followed by extranodal NK/T-cell lymphoma, nasal type, ALCL and AILT. The relatively frequent occurrence of extranodal NK/T-cell lymphoma,nasal type in Guangdong area is likely associated with the high incidence of Epstein-Barr virus infection there.