血管内大B细胞淋巴瘤的临床病理特征  

Clinicopathological features of intravascular large B-cell lymphoma

作　　者：普文静[1] 宋新兰[1] 孙振柱[1] PU Wen-jing;SONG Xin-lan;SUN Zhen-zhu(Department of Pathology,People's Hospital of Xinjiang Uygur Autonomous Region,Urumqi 830001,China)

机构地区：[1]新疆维吾尔自治区人民医院病理科,乌鲁木齐830001

出　　处：《诊断病理学杂志》2024年第7期648-652,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的 探讨血管内大B细胞淋巴瘤(IVLBCL)的临床病理学特征。方法 收集4例IVLBCL患者的临床及辅助检查资料,结合病理组织学及免疫组化特点,分析其临床病理特征。结果 患者男性3例,女性1例,年龄48~80岁。发生于前列腺的患者表现为进行性排尿困难;发生于右侧附睾的患者表现为阴囊肿大;发生于下肢的患者表现为下肢近端内侧红肿并触及硬结。镜下淋巴瘤细胞位于小血管腔内,细胞体积中等偏大,核浆比高,核仁明显,核分裂象可见。全部病例表达CD19、CD20、CD79a及MUM-1,部分病例表达BCL-6、CD5、CD30及CD10,同时表达BCL-2和C-MYC,Ki-67阳性指数高。结论 IVLBCL是一种罕见的侵袭性淋巴瘤,确诊需结合病理组织学形态及免疫组化,不同亚型临床表现和预后有差别,早诊断、含利妥昔单抗的联合化疗可延长生存期,BTK抑制剂靶向治疗可改善预后。Objective To investigate the clinicopathological features of intravascular large B-cell lymphoma(IVLBCL).Methods The clinical and auxiliary examination data of 4 cases of IVLBCL were collected, and the clinicopathological features were analyzed in combination with histopathological and immunohistochemical features. Results Three of the four patients were males and one was female, aged from 48 to 80 years. Patients with prostate disease presented with progressive dysuria;patients with right epididymis presented with enlargement of the scrotum;Patients with lower extremity involvement presented with bilateral redness and swelling of the inner thighs with palpated indurations. Microscopically, the lymphoma cells were located in the lumen of small blood vessels, with medium to large cell size, high nuclear to plasma ratio, prominent nucleoli, and visible mitotic figures. All cases were positive for CD19, CD20, CD79a and MUM-1, and some cases were positive for BCL-6, CD5, CD30 and CD10, as well as BCL-2 and C-MYC. Ki-67 index was high.Conclusions IVLBCL is a rare aggressive lymphoma, and its diagnosis should be based on histopathological and immunohistochemical features. Different subtypes have different clinical manifestations and prognosis. Early diagnosis and rituximab combined chemotherapy can prolong survival, and BTK inhibitor targeted therapy can improve prognosis.

关 键 词：血管内大B细胞淋巴瘤 临床病理特征 免疫组化 鉴别诊断 

分 类 号：R733[医药卫生—肿瘤]