ALK阳性大B细胞淋巴瘤一例并文献复习  

ALK-positive large B-cell lymphoma： a case report and review of the literature

作　　者：郗彦凤[1] 柴菲[3] 李静[1] 白玮[1] 白文启[2] 

机构地区：[1]山西省肿瘤医院病理科,太原030013 [2]山西省肿瘤医院结直肠肛门外科,太原030013 [3]山西医科大学研究生部,太原030013

出　　处：《白血病．淋巴瘤》2014年第12期740-742,共3页Journal of Leukemia & Lymphoma

基　　金：山西省自然科学基金（2011011038-1）

摘　　要：目的 探讨ALK阳性大B细胞淋巴瘤的临床病理特点.方法 回顾性分析1例ALK阳性大B细胞淋巴瘤患者的临床资料、组织病理学特征、免疫表型及荧光原位杂交（FISH）检测结果,并结合文献进行讨论.结果 患者为30岁男性,左颈淋巴结肿大.光学显微镜下观察淋巴结内瘤细胞呈片巢状,核圆而淡染,中位大核仁,胞质丰富,嗜双色性,呈免疫母细胞或浆母细胞分化特点.免疫组织化学结果示肿瘤细胞CD138＋、ALK＋、CD45RO＋、CD4＋、Perforin＋、CD117＋、Kappa＋、CD3-、CD20-、CD30-、CD38-、CD79a-、EMA-.FISH检测发现存在ALK基因的易位.患者手术后行CHOP方案化疗4个周期,4个月后病情进展,仍在继续接受治疗.结论 ALK阳性大B细胞淋巴瘤是弥漫大B细胞淋巴瘤的罕见亚型,具有独特的形态学、免疫表型和分子遗传学特点.Objective To study clinicopathologic features of ALK-positive large B-cell lymphoma.Methods The clinical data,histopathological characteristics,immunophenotype and fluorescence in situ hybridization （FISH） result of a patient with ALK-positive large B-cell lymphoma were analyzed and discussed combined with related literatures.Results A 30-years-old male patients with the left neck lymphadenectasis was studied.Histological evaluation revealed the tumor grew in sheets in the nodal,with round nuclei,dispersed chromatin,a single prominent central nucleolus and moderate amounts of eosinophilic to amphophilic cytoplasm.The neoplastic cells exhibited immunoblastic/plasmablastic morphology.Immunohistochemistry measurement showed that the tumor cells were marked positively by CD138,ALK-1,CD45RO,CD4,Perforin,CD117 and Kappa proteins,while negatively by CD3,CD8,CD20,CD30,CD38,CD57,CD79a,Pax-5,EMA and AE1/AE3 proteins.FISH test demonstrated the presence of ALK gene translocation.The patient was given 4 cycles of CHOP chemotherapy after surgery.However,the conditions deteriorated after 4 months.Now the patient continued to receive treatment.Conclusion ALK-positive large B-cell lymphoma represents a distinct variant of diffuse large B-cell lymphoma,and the tumor has special histological features along with a distinct immunophenotype and ALK gene rearrangement.

关 键 词：ALK阳性大B细胞淋巴瘤 免疫表型 原位杂交 荧光 鉴别诊断 

分 类 号：R733.1[医药卫生—肿瘤]