11例HIV阴性浆母细胞淋巴瘤(PBL)的临床特征分析

Clinical features of HIV-negative plasmablastic lymphoma (PBL):analysis of 11 cases

回顾性分析复旦大学附属中山医院2007年1月至12月确诊的11例HIV阴性浆母细胞淋巴瘤(plasmablastic lymphoma,PBL)患者的临床特点、治疗及预后,以积累对疾病的临床诊治经验。其中男性8例,女性3例,中位年龄45岁,60岁以上5例,均为结外受累起病,其中6例胃肠道受累首发。Ann Arbor分期Ⅳ期9例,Ⅲ期1例,另1例未能分期。所有患者均表达浆细胞特异性标记,CD20阴性8例,弱阳性2例,中位Ki67为75%。7例患者采用CHOP样方案化疗,其中1例联合硼替佐米治疗,2例获得完全缓解;另4例患者中1例采用EPOCH治疗,疾病进展后失访,1例采用EP方案治疗,1例采用来那度胺为主方案治疗,1例采用中药治疗,均因疾病进展导致死亡。中位无进展生存时间4.9个月,中位生存时间7.8个月。11例HIV阴性PBL患者均为高度侵袭性,男性及老年易发,胃肠道易受累,CHOP样方案疗效不佳,早期治疗可改善预后。

To accumulate more experience on diagnose and treatment of HIV-negative plasmablastic lymphoma(PBL),clinical characteristcs,treatment options and prognosis of 11 HIV negative PBL cases,including 8 males and 3 females,were analysed retrospectively from Jan.2007 to Dec.2017 in Zhongshan Hospital,Fudan University.The median age was 45 years,and 5 patients were over 60 years old.These patients were all extra-nodal involvement as origin,and 6 cases were gastrointestinal involvement.According to Ann Arbor staging,9 cases were atⅣstage,1 case was atⅢstage,and the left one could not be staged.All patients’tumor cells expressed plasma cell-specific markers.Eight cases were CD20 negative,and 2 cases were weakly positive.The median Ki67 was 75%.Seven patients were treated with CHOP-like regimen,and 1 case of them was also treated with bortezomib,2 cases of them achieved complete remission.Among the other 4 cases,1 case treated with EPOCH was lost after disease progression,1 case treated with EP regimen,1 case treated with lenalidomide-based regimen,and 1 case treated with Chinese medicine treatment were all dead.The median progression-free survival time was 4.9 months,and the median survival time was 7.8 months.Therefore,HIV-negative PBL in our center was highly invasive,male and old were susceptible,and the gastrointestinal tract was easily involved.The efficacy of CHOP-like regimen was frustrating,but early treatment might be helpful.