喉部鼻型NK/T细胞淋巴瘤一例临床分析并文献复习PhundreimayumRaeesAhmed1,谢敏2【摘要】目的研究喉部鼻型NK/T细胞淋巴瘤的临床表现、病理特点及免疫表型,提高对本病的认识。方法对收治的1例喉部鼻型NK/T细胞淋巴瘤进行介绍,并对2001年至2012年国内发表的该病报道进行回顾性分析。结果纳入8篇报道共10例确诊为喉部鼻型NK/T细胞淋巴瘤患者。患者多中年起病,临床表现多样,最常见首发症状为声音嘶哑(80%)。病变最常累及的部位为声带(87.5%),出现表面增生或浸润性肿块(85.7%)、粘膜肿胀(57.1%)。其镜下最常见表现为大片坏死组织,淋巴细胞增生活跃(55.6%)。免疫组化CD45RO、TIA-1、EBER1/2的阳性率均为100%,而CD56、粒酶B的阳性率为75%。结论喉部鼻型NK/T细胞淋巴瘤的临床表现多样,但其具有独特的病理、免疫学特点。【关键词】喉部;鼻型NK/T细胞淋巴瘤;临床分析ClinicalAnalysisof1CaseofLaryngealNasal-NK/T-CellLymphomawithLiteratureReviewPHUNDREIMAYUMRaeesAhmed1,XIEMin21DepartmentofRespiration,JawaharlalNehruHospital,Imphal,Manipur,India2DepartmentofRespiration,WestChinaHospitalofSichuanUniversity,Chengdu610041,Sichuan,ChinaAbstractObjective:ToimprovetheunderstandingofLaryngealNasal-NK/T-CellLymphomabyanalyzingitsclinical,pathologicalandimmunohistochemicalcharacteristics.Methods:Fulldescriptionof1caseoflaryngealnasal-NK/T-cellLymphomawasgiven,anddomesticreportsofitbetweenyear-2001to2012wereretrospectivelyanalyzed.Results:8reportswith10caseswerecollected.Mostpatientswereaffectedintheirmiddleage(90%)withonsetagevariedaround39.0(±10.6)yearsold,anddiedofperipheralrespiratoryfailure.Mostcommoninitialsymptomwashoarseness(80%).Patients’clinicalmanifestationsvaried,whichincludedhoarseness(80%),sorethroatorforeignbodysensation(70%),fever(50%),hemoptysis(40%)andsoon.Thediagnosisreliedonthebiopsyanditspathological,immunohistochemical,hybridizationinsitutests.Themostcommonlyinvolvedlesionwaslocatedinvocalcords(87.5%),whichpresentedwithsuperficialhyperplasiaorinvasivemass(85.7%),mucosalswelling(57.1%).Scatteredallotypedcellsburiedinthebackgroundofnecrotictissuewasmostcommonlyseenundermicroscope,withfoliated-locatedactivehyperplasiclymphocytes(55.6%).Inimmunohistochemistry,CD45RO,TIA-1,EBER1/2havethe100%positiverate,andCD56,GranzymeB75%,whileCD5,CD15,CD43,D57,CD68,CD79a100%innegativerate.Conclusions:Theclinicalmanifestationsoflaryngealnasal-NK/T-celllymphomavaried,butithasitsuniquepathologicalandimmunohistochemicalcharacteristics.KeyWords:NK/T-celllymphoma;Larynx;Clinicalanalysis0引言NK/T细胞淋巴瘤(naturalkillercell/T-celllymphoma,NK/T-celllymphoma)是WHO淋巴瘤分类(1997年)中新确立的一组特殊的外周T细胞与NK细胞淋巴瘤,并于2001年列入恶性淋巴瘤的新分类。NK/T细胞淋巴瘤包括:(1)鼻、鼻型NK/T细胞淋巴瘤;(2)肠病型T细胞淋巴瘤;(3)皮下脂膜炎样T细胞淋巴瘤;(4)肝、脾γδT细胞淋巴瘤[1-3]。其临床表现多样,诊断依赖病理及免疫组化,预后较差。喉部鼻型NK/T细胞淋巴瘤极少见。该病在南美及亚洲高发,且误诊率极高[1]。现将我院诊断的1例喉部鼻型NK/T细胞淋巴瘤报道如下,并对国内目前报道的喉部鼻型NK/T细胞淋巴瘤的临床表现、病理特点及免疫表型进行分析和总结。1病例报告男,59岁,因反复发热伴咳嗽咯痰1月余、咯血1天,且对头孢等抗生素效果不佳而入院。初起为反复发热,最高达39.9℃,不伴明显畏寒、寒战;咳嗽、咯少量白色泡沫痰、咽痛,伴声音嘶哑。于当地医院考虑“呼吸道感染”予“左氧氟沙星、哌拉西林他唑巴坦”等治疗后,症状无改善,体温波动于38.0℃左右,并于起病后一周渐出现进食、饮水呛咳,遂转至我院进一步诊治。入院查体:T39.0℃,P90次/分,R22次/分,BP85/55mmHg。咽部无明...