肝脾T细胞淋巴瘤临床病理学分析并文献复习  

Clinical and pathological analysis of hepatosplenic T-cell lymphoma and review of the literature

作　　者：张秀茹[1] 陈桂秋[2] 童春容[3] 卢一艳[1] 

机构地区：[1]航天中心医院病理科 [2]吉林大学中日联谊医院病理科 [3]北京道培医院血液科

出　　处：《中华临床医师杂志（电子版）》2012年第2期105-108,共4页Chinese Journal of Clinicians(Electronic Edition)

摘　　要：目的探讨肝脾T细胞淋巴瘤的临床病理特征。方法报道1例罕见的肝脾T细胞淋巴瘤的临床资料、病理学诊断依据并行文献复习。结果本例肝脾T细胞淋巴瘤患者肝脾明显增大,伴有反复发热,全血细胞减少。脾组织印片示:85%为幼稚淋巴细胞。病理示:脾结构破坏,瘤细胞呈窦性浸润。瘤细胞免疫表型CD3+++,LCA+,PAX5残存正常B淋巴细胞+,CD30-,CD79a-,CD20-,CD21-,CD10-,CD68少量+,CD34血管+,Ki-67+>70%,Bcl-2++,P53-。EBV-DNA+,定量4×103拷贝/106PBNC;脾流式细胞检测示:CD4,CD8,CD45RA,CD5均-,而CD7,CD3,CD2,TCRγδ-1,HLA-DR,CD38均+,提示γδT淋巴瘤;基因重排结果显示:IgH-,TCRγ+;染色体核型分析示:46,X,-X,i(7q),+8[20]/46,XX[3]。结论肝脾T细胞淋巴瘤是较为罕见的外周T细胞淋巴瘤,预后较差,可通过多种分子生物学相关辅助检查快速准确地协助诊断和鉴别诊断。[ Abstract ] Objective To study the clinical pathological features （HSTCL）. Methods In this article we report the clinical material and of hepatosplenic T-cell lymphoma pathology dignosis of one case of hepatosplenic T-cell lymphoma and review the literature. Results The femal patient was hospitalized with significantly increased size of liver and spleen, accompanied by recurrent fever, pancytopenia. Spleen imprint showed： 85% of the cells were naive lymphocytes. Pathology showed：spleen structure was damazed,tumor cells showed sinusinfiltration. Tumor cell immune phenotype ： CD3 ＋ ＋ ＋ , LCA ＋ , PAX5 normal B lymphocyte ＋ , CD68 partial ＋,CD34 blood vessels ＋ ,Ki-67 ＋ 〉70% ,Bcl-2 ＋ ＋ ,P53 -. EBV-DNA ＋ ,quantitative 4 × 10^3 copies/10^6 PBNC.By FC the diagnostic HSTCL immunophenotype was CIM - , CD8 - , CIM5RA - , CD5 - , hut CD7 ＋ , CD3 ＋ , CD2 ＋ , TCRγδ ＋ , HLA-DR ＋ , CD38 ＋. Gene rearrangement result showed that ：IgH - , TCRγ＋. Chromosome karyotype analysis showed：46,X, - X,i（7q）, ＋ 8 [20]/46,XX[3]. Conclusions Hepatosplenie T-cell lymphoma is a relatively rare peripheral T-cell lymphoma（peripheral T cell lymphoma, PTCL）, the prognosis is poor. Variety of molecular biology-related examinations can be used to assist the diagnosis and differential diagnosis.

关 键 词：淋巴瘤 T细胞 免疫表型分型 流式细胞术 基因重排 染色体核型分析 

分 类 号：R735[医药卫生—肿瘤]