Objective: To evaluate the clinicopathologic characteristics and outcomes of HIV-negative plasmablastic lymphoma (PBL) . Methods: Medical records of 15 patients diagnosed with HIV-negative PBL in Changhai Hospital between January 2013 and August 2019 were reviewed, and clinicopathologic characteristics and outcomes were analyzed. Results: Median age was 59 years (range: 17-69) . All patients had extranodal involvement. According to the Cotswolds-modified Ann Arbor staging system, 1 (6.7%) , 2 (13.3%) , 3 (20.0%) , and 9 (60.0%) patients were classified as at Ⅰ,Ⅱ,Ⅲ and Ⅳ, respectively. Plasmablast and immunoblast proliferations were typical manifestations of PBL. Immunohistochemical staining showed tumor cells were diffusely positive for plasma cell markers CD38, CD138, and Mum-1, while negative for B cell markers CD20, CD10, PAX-5, and BCL-6. Median Ki-67 index was 80% (70%-90%) . Epstein-Barr virus-encoded RNA (EBER) expression was detected in 3 patients, and 1 of them was positive. All patients received chemotherapy, 80% combined with bortezomib as the first line, and responses were observed in 8 patients (6 complete and 2 partial responses) . Median progression-free survival (PFS) and overall survival (OS) were 6.8 (95% CI 2.5-11.1) months and 17.9 (95% CI 5.6-30.2) months, the 3-year PFS and OS rates were 21.2% (95% CI 1.4%-56.8%) and 38.5% (95% CI 12.0%-65.0%) , respectively. Conclusion: HIV-negative PBL with high invasiveness is extremely prone to extranodal involvement and most patients were at the advanced stage. Patients receiving an intensive therapy combined with bortezomib and bridged autologous stem cell transplantation may improve long-time survival.
目的： 分析HIV阴性的浆母细胞淋巴瘤（PBL）患者的临床病理特征及转归。 方法： 回顾性分析海军军医大学附属长海医院2013年1月至2019年8月诊断及治疗的15例HIV阴性PBL患者的临床病理资料及随访结果。 结果： 15例HIV阴性PBL患者中男8例，女7例，中位年龄59（17~69）岁，均无明确的免疫抑制状态。所有患者均出现结外受累，Ann Arbor-Cotswolds分期Ⅰ、Ⅱ、Ⅲ、Ⅳ期分别为1例（6.7%）、2例（13.3%）、3例（20.0%）、9例（60.0%）。病理形态表现为类似免疫母细胞的大肿瘤细胞弥漫性增生，免疫组化示肿瘤细胞主要表达浆细胞标志CD38、CD138、Mum-1，B细胞标志CD20、CD10、PAX-5、BCL-6少见，Ki-67中位数为80%（70%~90%）。3例患者行EBER原位杂交检测，1例阳性。15例患者均接受化疗，80%（12/15）联合硼替佐米作为一线治疗。最佳疗效6例完全缓解（其中2例分别于治疗后4.9和26.1个月疾病进展），2例部分缓解（分别于治疗后3.0和6.8个月疾病进展），4例疾病进展，3例无法评估。中位随访时间30.3（4.8~61.1）个月，11例患者中位无进展生存（PFS）期6.8（95%CI 2.5~11.1）个月，预计3年PFS率为21.2%（95%CI 1.4%~56.8%）。所有患者中位总生存（OS）期为17.9（95%CI 5.6~30.2）个月，预计3年OS率为38.5%（95%CI 12.0%~65.0%）。 结论： HIV阴性PBL侵袭性强，结外受累常见，多数患者诊断时分期较晚。通过以硼替佐米为基础的强化疗方案序贯自体造血干细胞移植可望获得长期生存。.
Keywords: Autologous stem cell transplantation; Bortezomib; Plasmablastic lymphoma.