Han X, Hu L X, Ouyang M Q, Duan M H, Zhou D B
Department of Hematology, Peking Union Medical College Hospital, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2017 Apr 14;38(4):290-294. doi: 10.3760/cma.j.issn.0253-2727.2017.04.006.
To deepen the knowledge of HIV-negative plasmablastic lymphoma (PBL) . 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. 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. 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.
为加深对HIV阴性浆母细胞淋巴瘤（PBL）的认识。收集了1997年1月至2015年5月在北京协和医院确诊的8例HIV阴性PBL患者的病历，并分析了这些患者的临床特征和预后。这8例患者均被诊断为HIV阴性PBL，8例患者中3例为男性，其余为女性。中位年龄为60（43 - 80）岁。这些患者中，4例有潜在的免疫抑制状态。所有患者均有结外受累，根据Ann Arbor分期，其中6例为Ⅳ期，5例有骨髓受累。所有患者CD38和CD138均弥漫性阳性，而包括PAX - 5和Bcl - 6在内的B细胞标志物阳性率相对较低。8例患者中有5例检测了EBV - DNA，均为阴性。7例接受化疗并定期随访的患者中位随访时间为36（11 - 57）个月，中位无进展生存期（PFS）为15（6 - 52）个月，中位总生存期为36（2 - 52）个月。这些患者中，4例接受了化疗联合硼替佐米治疗，3例有效，但似乎难以维持长期疗效，治疗后2、9和21个月疾病进展。2例Ⅰ - Ⅱ期患者治疗有效，无疾病进展且存活，5例Ⅳ期患者疗效不可持续，中位PFS为10（2 - 21）个月，中位总生存期为12（6 - 52）个月。HIV阴性PBL在老年患者中相对常见，临床侵袭性高，极易发生结外受累，尤其是骨髓受累。PBL的免疫表型更类似于浆细胞瘤。诊断时处于晚期的患者预后较差。
