Objective: To investigate clinic-pathological characteristics, diagnosis, treatment and prognosis of intravascular large B cell lymphoma (IVLBCL) in China. Methods: Clinical and pathological records were analyzed from 12 IVLBCL patients diagnosed between Jan 2010 to Jun 2016. Kaplan-Meier method was used to estimate overall survival (OS), and univariate analysis was performed to identify prognostic factors. Results: A series of 12 patients with IVLBCL (median age, 53.8 years; range, 32-76 years; 6 males and 6 females) was reviewed. Fever was the most common symptom (10/12), respiratory symptoms (cough, pleural effusion, dyspnea, 50%) and hemophagocytic lymphohistiocytosis (50%) were frequently observed, and only 12 patients had neurological symptom. All patients had elevated lactic dehydrogenase and serum ferritin. International Prognostic Index score was high in 75% of total patients. All patients had extra-nodal involved, pulmonary (6/12) and bone marrow (4/12) were frequently involved. Large lymphoid cells within vessel lumina or sinuses were observed in all patients. These cells were large, with scant cytoplasm, vesicular nuclei, and one or more nucleoli, and the structures of vessels and sinus were reserved. CD20 and CD79a were positive in all cases. 11patients received rituximab combined CHOP regimen chemotherapies, overall response rate (ORR) was 90.1%, and complete response rate was 66.7%. Median survival time and median progression time were not reached after a median follow-up of 20 months. Univariate analysis revealed that no clinical characters were associated with OS. Conclusion: As a rare variant of DLBCL, IVLBCL presented with pulmonary involved frequently, and trans-bronchial lung biopsy had good positive rates. Rituximab contained chemotherapy was the backbone for IVLBCL.
目的： 提高对血管内大B细胞淋巴瘤（IVLBCL）的认识。 方法： 回顾性分析北京协和医院自2010年1月至2016年6月收治的12例IVLBCL患者的临床资料。 结果： ①12例患者中，男6例，女6例，中位年龄为58.5（32~76）岁。临床表现：发热最为常见（10/12），其次为呼吸系统症状（咳嗽、呼吸困难、胸腔积液等，6/12）、噬血细胞综合征（6/12）、神经系统症状（1/12）；所有患者均有LDH升高（367~1 700 U/L）、铁蛋白升高（805~1 527 μg/L）、结外器官受累（肺6例，骨髓3例，脑2例，软脑膜、肾上腺、副鼻窦、胆囊、甲状腺各1例）。②组织病理特征：光镜下可见分布于小血管内的单个或小簇状淋巴瘤细胞，毛细血管或血窦结构得以保留，免疫组化显示CD20、CD79a等B淋巴细胞标志阳性。③疗效及预后：11例患者接受CHOP或R-CHOP方案化疗，其总反应率为90.1%，完全缓解率为66.7%。患者平均随访20（1~40）个月，中位生存期及中位无进展生存期尚未达到。单因素Cox回归分析未能发现年龄、分期、IPI等临床参数与预后相关。 结论： IVLBCL临床罕见，出现肺部受累比例高，肺活检有较高阳性率；R-CHOP方案目前仍是最主要的治疗方案。.
Keywords: Clinical analysis; Lymphoma, B cell; Pulmonary involved; Treatment outcomes.