作　　者： (曾英）; (马强）; (赵连花）; (杜娟）; (马瑜）; (钟鹏）; (林俐）; (付萍）; (肖华亮）;

机构地区： 第三军医大学大坪医院野战外科研究所,重庆400042

出　　处： 《诊断病理学杂志》 2017年第8期581-585,共5页

摘　　要： 目的探讨血管内大B细胞性淋巴瘤(IVLBCL)的临床病理学特征及鉴别诊断要点。方法收集3例IVLBCL临床及影像学资料,观察其病理学形态及免疫表型并复习相关文献,探讨该肿瘤的病理诊断与鉴别诊断要点。结果患者男性2例,女性1例,年龄51~78岁。2例发生于双侧肾上腺,1例发生于颅内右侧顶叶。发生在肾上腺者表现为腰背部疼痛或不适;发生于右侧顶叶者表现为头昏、头痛。镜下观察3例形态学表现相似,瘤细胞位于小血管腔内,大血管也可见累及,瘤细胞大,核空泡状,核仁明显,核分裂易见,可见纤维素性血栓。免疫组化:CD20、CD79a、PAX-5、bcl-6、MUM-1和bcl-2(+),CK、CD3、CD5、CD30、CD10、EBER、Syn、SALL4、OCT3/4、CD117和PLAP(-),c-Myc核阳性比例为10%~20%,3例Ki-67阳性指数分别为30%、40%和80%。结论血管内大B细胞性淋巴瘤是一种罕见的结外弥漫性大B细胞性淋巴瘤,需与其他小细胞恶性肿瘤鉴别,其确诊需结合组织病理学及免疫组化。该瘤高度恶性和高度侵袭性,对化疗不敏感,由于临床表现不特异,早期诊断困难。多数患者诊断后短期死亡,预后很差,因此临床应引起警惕,尽早诊断、积极联合化疗可延长生存期。 Objective To investigate clinicopathologic features and differential diagnosis of intravascular large B-cell lymphoma（ IVLBCL）. Methods The clinical and radiological data were collected in three cases of IVLBCL,their pathological and immunohistochemical features were analyzed,with discussion of the pathological diagnosis and differential diagnosis based on related literature. Results The age of the patients at diagnosis ranged from 51 to 78 years; two cases were male and one case was female. The two lesions were located in the adrenal glands,and one lesion was located in the right parietal lobe of the brain. Clinical manifestation of the patients in the adrenal gland was back pain or discomfort; the patient in the right parietal lobe showed dizziness and headache. Microscopic observation showed morphological findings were similar in the 3 cases： tumor cells located in small vascular lumina,and large blood vessels could also be involved.Tumor cells were large, nuclear vacuoles, and prominent nucleoli. High mitosis and fibrin thrombus were seen.Immunohistochemistry showed that the tumor cells were positive for CD20,CD79 a,PAX-5,Bcl-6,MUM-1 and bcl-2; but negative for CK,CD3,CD5,CD30,CD10,EBER,Syn,SALL4,OCT3/4,CD117 and PLAP; C-Myc positive nucleus ratio was 10% ~ 20%; Ki-67 proliferation index was 30%,40%,and 80%,respectively. Conclusion IVLBCL is a rare variant of diffuse large B cell lymphoma. Differential diagnosis includes other small cell malignant tumors. The diagnosis should be combined with histopathological changes and immunohistochemical phenotype. The tumor is highly malignant and aggressive,and not sensitive to chemotherapy; most patients die in a short time after diagnosis,and so prognosis is poor. Clinical manifestations are nonspecific and early diagnosis is difficult. Clinicians should arouse vigilance,try their best to diagnose early and chemotherapy can prolong the patient’s survival time.

关 键 词： 血管内大 细胞性淋巴瘤 免疫组化 临床病理特征 鉴别诊断