Objective: To deepen the knowledge of HIV-negative plasmablastic lymphoma (PBL) . Methods: Medical records from 8 HIV-negative PBL patients diagnosed in Peking Union Medical College Hospital from January 1997 to May 2015 were collected, and the clinical features and prognosis of these patients were analyzed. Results: All of these 8 patients were diagnosed as HIV-negative PBL, 3 of 8 patients were males, and others were female. The median age was 60 (43-80) year. Among these patients, 4 cases had underlying immunosuppressive state. These patients all had extra-nodular involvement, and 6 cases of them were at stage Ⅳ according to Ann Arbor Staging, 5 patients had bone marrow involvement. CD38 and CD138 were diffusely positive for all patients, while the positive rate of B cell marker including PAX-5 and Bcl-6 were relative low. 5 of 8 patients had been detected for EBV-DNA, and all of them were negative. The median follow-up for the 7 patients receiving chemotherapy and regular follow-ups was 36 (11-57) months, the median progression-free survival (PFS) was 15 (6-52) months, and the median overall survival was 36 (2-52) months. Among these patients, 4 cases had received chemotherapy combined with Bortezomib, showing 3 cases of effective, but it seems to be difficult to keep the long term efficacy, and disease progression occurred in 2, 9, and 21 months after treatment. 2 patients at stageⅠ-Ⅱ were treated effectively, without disease progression and survival, 5 patients at stage Ⅳacquired the efficacy unsustainably, with a median PFS of 10 (2-21) months and a median overall survival of 12 (6-52) months. Conclusion: HIV-negative PBL is relatively prevalent in elderly patients, and presenting with high invasiveness in clinical, extremely prone to extra-nodular involvement, especially the bone marrow. The immunophenotype of PBL is more resemble to that of plasmacytoma. Patients who were in late stage at diagnosis show poor prognosis.
目的： 提高对人免疫缺陷病毒（HIV）阴性浆母细胞淋巴瘤的认识。 方法： 回顾性分析北京协和医院1997年1月至2015年5月确诊的8例HIV阴性浆母细胞淋巴瘤患者的临床资料，分析其临床特征及转归。 结果： 8例HIV阴性浆母细胞淋巴瘤中男3例，女5例，中位年龄60（43~80）岁，其中4例存在导致免疫功能低下的疾病或状态。8例患者均有结外受累，2例Ann Arbor分期为Ⅰ~Ⅱ期，6例为Ⅳ期，其中5例有骨髓受累。所有患者均弥漫表达CD38和CD138，B细胞标志包括PAX-5及Bcl-6少见。5例患者进行EBV-DNA检测，均为阴性。接受化疗并规律随访的7例患者中位随访36（11~57）个月，中位无进展生存时间为15（6~52）个月，中位总生存时间为36（2~52）个月；其中4例采用了硼替佐米联合化疗，3例有效，但疗效难以维持，分别于治疗后2、9、21个月疾病进展。2例Ⅰ~Ⅱ期患者均治疗有效，未出现疾病进展，持续存活；5例Ⅳ期患者化疗后虽然有效，但疗效难以维持，中位总生存时间仅12（6~52）个月，中位无进展生存时间仅10（2~21）个月。 结论： 该组HIV阴性浆母细胞淋巴瘤患者以中老年为主，临床呈现高侵袭性，均出现结外（尤其是骨髓）受累，其免疫表型与浆细胞瘤较为接近，分期较晚的患者预后不良。.
Keywords: Clinical characteristics; Plasmablastic lymphoma; Treatment outcome.