肝脾γδ T细胞淋巴瘤及其与EB病毒感染的关系

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1、肝脾γδT细胞淋巴瘤及其与EB病毒感染的关系作者：高晓宁　唐锁勤　刘英　王建文【摘要】　　为探讨肝脾γδT细胞淋巴瘤的临床及病理特点及其与EB病毒感染的关系，对1例9岁女性肝脾γδT细胞淋巴瘤的患者进行了临床观察，采集骨髓液涂片行瑞氏染色，骨髓活检和肝脏活检的组织切片后常规化学染色及免疫组织化学标记，用EB病毒寡核苷酸探针(EBER)原位杂交。结果显示，患儿长期发热、贫血、血小板减低，肝脾进行性肿大，伴慢性活动性EB病毒感染，血清乳酸脱氢酶、铁蛋白呈进行性增高，多形性小淋巴细胞浸润骨髓基质，肝内增生浸润

2、的淋巴细胞强表达T细胞标记物CD3和活化细胞毒性标记物粒酶B，EB病毒mRNA阴性。结论：肝脾γδT细胞淋巴瘤具有独特的临床和组织病理特征，肝脏和脾脏和骨髓病理检查及免疫表型分析有助于诊断；EB病毒感染可能是γδT细胞发生恶性转化后的晚期事件。【关键词】肝脾γδT细胞淋巴瘤　EB病毒感染　淋巴瘤HepatosplenicγδTCellLymphomaandItsRelationshipwithEpstEin-BarrVirusInfectionAbstractToexploretheclinicalan

3、dpathologicalcharacteristicsofhepatosplenicγδT-celllymphomaanditsrelationshipwithEpstEIn-Barrvirusinfection,theclinicalfeaturesofa-year-oldgirlwithhepatosplenicγδT-celllymphomawereinvestigated,thesmearsofbonemarrowwasstainedwithWright′sstain,biopsiesofbo

4、nemarrowandliverspecimenwereembeddedinplasticandslicedaboutμminthicknessandroutinelystainedwithHEstaining,theimmunohistochemicalstainingwasusedtomarkthetumorcells,andEBERprobeswereusedtodetectEpstein-BarrvirusRNA.Theresultsshowedthatthegirlpresentedwithp

5、rolongedfever,anemia,thrombocytopenia,hepatosplenomegaly,chronicactiveEpstein-Barrvirusinfection,andelevatedlevelsofserumferritinandlactatedehydrogenase.Bonemarrowaspiraterevealedtheinfiltrationofatypicallymphocytesinthebonemarrowstroma.Theliverbiopsyspe

6、cimenrevealedtheinfiltrationoflymphocytesinthesinusoids,whichwaspositivefortheT-cellassociatedmarkerCDandactivatedcytotoxicity-associatedmarkergranzymeB.In-situhybridizationanalysiswithEBERprobesrevealedthattheabove-mentionedcharacteristicswerenegativein

7、neoplasticcells.ItisconcludedthathepatosplenicγδT-celllymphomaisadiseasewithdistinctiveclinical,histopathologic,andphenotypiccharacteristics.Hepaticand/orsplenicand/orbonemarrowbiopsywithcombinedphenotypeisbeneficialtodiagnosis.Epstein-Barrvirusinfection

8、islateeventinvolvinganalreadytransformedgammadeltaT-cellclone.KeywordshepatosplenicγδTcelllymphoma;Epstein-Barrvirusinfection;lymphoma肝脾γδT细胞淋巴瘤是γδT细胞克隆性增殖导致的临床过程呈侵袭性的恶性疾病，由Farcet等［1］在1990年首先描述，他们通过仔细分析以前的2例主要浸润肝脏和脾脏的外周γδT