血管内外周T细胞淋巴瘤的临床病理特征  

Clinicopathological features of intravascular peripheral T-cell lymphoma

作　　者：管雪 杨亦宸 祁昱 巩文辰 徐晓莹 王雅蕾 郭玉虹 罗烨 孙琳 付凯 孟斌 Guan Xue;Yang Yichen;Qi Yu;Gong Wenchen;Xu Xiaoying;Wang Yalei;Guo Yuhong;Luo Ye;Sun Lin;Fu Kai;Meng Bin(Tianjin Medical University Cancer Institute and Hospital,National Clinical Research Center for Cancer Key Laboratory of Cancer Prevention and Therapy,Tianjin 300060;Tianjin’s Clinical Research Center of Cancer,Tianjin 300060,China;Department of Pathology,Tianjin Medical University Cancer Institute and Hospital Tianjin 300060,China;Department of Pathology,Roswell Park Cancer Institute,Buffalo,New York)

机构地区：[1]天津医科大学肿瘤医院病理科,国家肿瘤临床医学研究中心,天津市肿瘤防治重点实验室,天津市恶性肿瘤临床医学研究中心,天津医科大学肿瘤医院中美淋巴血液肿瘤诊治中心,300060 [2]天津医科大学肿瘤医院中美淋巴血液肿瘤诊治中心,美国罗斯威尔帕克癌症研究所病理科

出　　处：《中华血液学杂志》2021年第7期583-590,共8页Chinese Journal of Hematology

摘　　要：目的总结血管内T细胞和NK细胞淋巴瘤的临床病理特点,增加对此类疾病的认识,以减少漏诊、误诊。方法回顾性分析1例原发于淋巴结内的血管内外周T细胞淋巴瘤,非特指型(IVPTCL,NOS)患者的临床及病理学特点,并复习相关文献资料。结果患者,男性,66岁,PET-CT示全身多发淋巴结肿大,活检示正常淋巴结结构消失,淋巴滤泡部分破坏。高倍镜下显示大量血管弥漫增生、扩张,其内见异型淋巴样细胞团局限生长于管腔内,部分浸润大血管壁,细胞中等偏大,胞质中等,核不规则,可见单个或多个核仁,染色质呈凝聚状,部分空亮,核分裂象可见。肿瘤细胞表达CD3、CD43、CD8、GrB、TIA-1及穿孔素等;原位杂交EBV-EBER(-);TCR基因重排检测(+)。予CHOP联合西达本胺方案化疗,患者不足2个月因感染、心肺功能衰竭死亡。检索相关文献,收集到有明确分型的血管内T细胞和NK细胞淋巴瘤56例,其中鼻型结外NK/T细胞淋巴瘤47例(男性27例,女性20例),间变性大细胞淋巴瘤8例(男性3例,女性5例),仅有1例为原发于脑组织的IVPTCL,NOS。本例为第2例报道的IVPTCL,NOS,且为第1例原发于淋巴结内的病例报道。结论血管内T细胞和NK细胞淋巴瘤为高度侵袭性疾病,目前尚无有效治疗方案。此病累及淋巴结的报道少,尚需积累更多病例进一步研究。Objective To summarize the clinical and pathological features of intravascular NK and T cell lymphoma for better understanding of such disease to reduce misdiagnosis and miss-diagnosis.Methods Clinical and pathological features were analyzed retrospectively in one case of intravascular peripheral T-cell lymphoma,not otherwise specified(IVPTCL,NOS),with literatures review.Results The case presented in this study was a 66-year-old man.PET/CT scan showed multiple lymph nodes enlargement throughout the body.Normal lymph node structure could not be observed by tissue biopsy,while lymph follicles were partially disrupted.High-power light microscope revealed a large number of blood vessels with diffuse proliferation and dilation,where atypical lymphoid cell mass was restricted in the lumen and partially infiltrated the large blood vessel wall.These tumor cells were medium to large with moderate cytoplasm.The nucleus was irregular,single or multiple nucleoli could be seen,chromatin was condensed,some were empty and bright,and mitotic figures could be seen.Immunohistochemical staining showed that the neoplastic cells were positive for expression of CD3,CD43,CD8,GrB,TIA-1 and perforin.EBER in situ hybridization result was negative.Polymerase chain reaction test identified a clonal gene rearrangement of T-cell receptorγ.The patient was treated with CHOP in combination with chidamide,but died of infection and cardiopulmonary failure within 2 months.56 cases of intravascular NK/T cell lymphoma with definite classification were collected from relevant literatures,including 47 cases with nasal type of extranodal NK/T cell lymphoma(27 were male and 20 were female),8 cases with anaplastic large cell lymphoma(3 males and 5 females),and only one case with de nova IVPTCL,NOS in brain.We report the second case of IVPTCL,NOS,and notably originated from lymph node for the first time.Conclusions Intravascular NK/T cell lymphoma is a highly aggressive disease with no effective treatment at present.Involvement of Lymph node has rarely be

关 键 词：外周T细胞淋巴瘤 非特指型 血管内淋巴瘤 淋巴结 病理诊断 

分 类 号：R732.2[医药卫生—肿瘤]