Fu W J, He M X, Huang A J, Gao L, Lu G H, Chen J, Chen L, Ni X, Zhang W P, Wang J M, Yang J M
Department of Hematology, Institute of Hematology, Changhai Hospital Affiliated to Navy Military Medical University, Shanghai 200433, China.
Department of Pathology, Changhai Hospital Affiliated to Navy Military Medical University, Shanghai 200433, China.
Zhonghua Xue Ye Xue Za Zhi. 2020 Jun 14;41(6):456-461. doi: 10.3760/cma.j.issn.0253-2727.2020.06.004.
To evaluate the clinicopathologic characteristics and outcomes of HIV-negative plasmablastic lymphoma (PBL) . 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. 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% 2.5-11.1) months and 17.9 (95% 5.6-30.2) months, the 3-year PFS and OS rates were 21.2% (95% 1.4%-56.8%) and 38.5% (95% 12.0%-65.0%) , respectively. 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.
评估HIV阴性浆母细胞淋巴瘤(PBL)的临床病理特征及预后。回顾了2013年1月至2019年8月在长海医院确诊为HIV阴性PBL的15例患者的病历,并分析其临床病理特征及预后。中位年龄为59岁(范围:17 - 69岁)。所有患者均有结外受累。根据Cotswolds改良的Ann Arbor分期系统,分别有1例(6.7%)、2例(13.3%)、3例(20.0%)和9例(60.0%)患者被分类为Ⅰ期、Ⅱ期、Ⅲ期和Ⅳ期。浆母细胞和免疫母细胞增殖是PBL的典型表现。免疫组化染色显示肿瘤细胞浆细胞标志物CD38、CD138和Mum - 1弥漫性阳性,而B细胞标志物CD20、CD10、PAX - 5和BCL - 6阴性。中位Ki - 67指数为80%(70% - 90%)。3例患者检测到爱泼斯坦 - 巴尔病毒编码RNA(EBER)表达,其中1例为阳性。所有患者均接受化疗,80%的患者一线联合硼替佐米,8例患者观察到反应(6例完全缓解和2例部分缓解)。中位无进展生存期(PFS)和总生存期(OS)分别为6.8(95% 2.5 - 11.1)个月和17.9(95% 5.6 - 30.2)个月,3年PFS率和OS率分别为21.2%(95% 1.4% - 56.8%)和38.5%(95% 12.0% - 65.0%)。侵袭性高的HIV阴性PBL极易发生结外受累,大多数患者处于晚期。接受联合硼替佐米的强化治疗并进行桥接自体干细胞移植的患者可能改善长期生存。