Objective: To evaluate the clinical features and outcomes of HIV-negtive plasmablastic lymphoma (PBL). Methods: Nine patients with HIV-negtive PBL were diagnosed and treated between January 2006 and January 2016. The clinical and follow-up data were analyzed retrospectively. Results: The median age was 56 years (range 30-77 years) with a male-to-female ratio of 2∶1. Nobody had underlying diseases associated with immunosuppression. Primary extra nodal diseases were observed in 7 cases and only 1 patient had oral involvement. Two patients were in earlystage and 7 in advanced stage by the Ann Arbor stage system. Ki-67 index was 60%-90% in the 9 cases, and 80% or higher in 7 cases. Epstein-Barr virus-encoded RNA expression (EBER) was detected in 4 cases, and 2 of them were positive. Chemotherapy was documented in 9 patients, from which 8 received the cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-like regimens as the first-line chemotherapy and responses were observed in 5 (1 complete, 4 partial responses). Three elderly patients were treated with CHOP combined with thalidomide, and 2 of them achieved partial responses. One patient, failed three chemotherapy regimens, accepted thalidomide combined with etopside and achieved stable disease for 10 months. One patient with early stage had disease-free survival of 61.9 months after treatment. The other eight patients experienced recurrence or progression after the first-line chemotherapy, and 6 of them died of disease progression within 2 years after the diagnosis. Conclusion: The HIV-negative PBL patients in this study did not have an apparent association with immunosuppression. Primary extra nodal diseases were common, but only 1 patient had oral involvement. Most patients had advanced stage and poor prognosis. Effectiveness of thalidomide in the treatment of PBL deserves further investigation.
目的: 评价人免疫缺陷病毒（HIV）阴性的浆母细胞淋巴瘤（PBL）的临床特征。
方法: 回顾性分析中国医学科学院肿瘤医院2006年1月至2016年1月收治的具有完整临床资料的9例HIV阴性PBL患者临床资料。
结果: 9例HIV阴性PBL患者中，男6例，女3例，中位年龄为56 (30~77）岁，均不存在免疫抑制相关的基础疾病。7例原发于结外，其中1例（例4）口腔受侵。Ann Arbor分期：Ⅱ期2例，Ⅳ期7例；9例患者Ki-67指数为60%~90%，≥80％者7例；4例患者进行EB病毒编码的小RNA（EBER）检测，2例EBER阳性。9例患者均接受化疗，8例一线采用CHOP（环磷酰胺＋表阿霉素＋长春新碱＋泼尼松）样方案，仅5例获得缓解（完全缓解1例，部分缓解4例）。其中3例老年患者采用CHOP联合沙利度胺方案，2例获得部分缓解。1例3个化疗方案失败的患者（例7）四线接受沙利度胺＋足叶乙甙治疗，疗效评价为疾病稳定，并维持10个月。1例早期患者（例4）无病生存61.9个月，其余8例一线治疗后均出现复发或进展，其中6例因疾病进展在诊断后2年内死亡。
结论: 本组HIV阴性PBL患者均无明确的免疫抑制因素。原发结外病变常见，但仅1例口腔受侵。多数患者分期晚、治疗效果差。沙利度胺用于PBL的治疗值得进一步研究。