15例HIV阴性浆母细胞淋巴瘤患者临床病理特征及转归

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.