HIV阴性浆母细胞性淋巴瘤2例报道并文献复习

HIV negative plasmablastic lymphoma: a report of two cases and review of literature

目的探讨浆母细胞性淋巴瘤(PBL)的病因、诊断和鉴别诊断、治疗及预后。方法对2009-2010年收治的2例HIV阴性PBL患者的临床资料进行回顾性分析,并复习国内外相关文献,总结临床诊治经验。结果 2例患者均诊断明确,HIV阴性,分别以淋巴结肿大和肋骨病变起病,均表现弥漫性大细胞淋巴瘤的形态学特征,同时表现出典型的浆细胞表型,免疫组化示CD38、CD138、CD79a阳性,低表达或不表达CD20、PAX-5。PET-CT均可见多部位(如淋巴结、骨骼)受累。CHOP样方案及ESHAP、IGVE等强化治疗方案的疗效不佳,缓解后复发快。2例患者从发病至死亡时间分别为2和7个月。结论 PBL罕见,恶性程度高,预后极差。患者对化疗药物的敏感性低,生存期短,目前此病尚无标准化疗方案。

Objective To investigate the origin,diagnosis,differential diagnosis,treatment,and prognosis of plasmablastic lymphoma（PBL）.Methods Based on the retrospective analysis of the clinical data of two patients with PBL admitted to our department from 2009 to 2010,followed by a review of related literature in China and abroad,we summarized our experience of the diagnosis and treatment of PBL.Results Two patients as reported in this paper were explicitly diagnosed to be HIV negative（–）： one presented enlarged lymph nodes,whereas the other presented lesions in the ribs.Both displayed the same morphology characteristics of diffused large cell lymphoma.They also exhibited the typical plasma cell phenotype.Immunohistochemical analysis revealed that the cells stained positively for CD38,CD138,and CD79a,and stained poorly or even negatively for CD20 and PAX-5.PET-CT scans demonstrated that it involved several body parts,such as lymph nodes and bones.CHOP regime,ESHAP,IGVE,and other intensive treatment regimes showed no satisfactory effect.Relapse appeared quickly after a brief remission,and both patients died 2 and 7 months,respectively,after the diagnosis.Conclusions We can conclude that PBL is very rare,but with high malignancy and poor prognosis.All PBL patients present low sensitivity to chemotherapy drugs and exhibit short survival rates.There is no standard chemotherapy regimen for PBL.