浆母细胞性淋巴瘤9例临床病理特征分析

Plasmablastic lymphoma:clinical and pathological features of 9 cases

目的 探讨浆母细胞性淋巴瘤(plasmablastic lymphoma, PBL)的临床病理特征、治疗、预后和鉴别诊断。方法 收集9例PBL的临床资料,采用HE、免疫组化EnVision法染色,应用原位杂交法检测EB病毒,运用PCR法检测B淋巴细胞基因重排,分析其临床病理特征并复习相关文献。结果 9例患者中,男性7例,女性2例,中位年龄54岁。鼻腔和鼻窦2例,淋巴结2例,牙龈1例,扁桃体1例,皮肤1例,结肠、肛管各1例。9例中HIV阳性4例,皮肤病变为浆细胞样细胞,其余部位为浆母细胞样/免疫母细胞样。免疫表型:CD138、MUM1、C-myc均阳性,CD20、CD3和CD5均阴性,CD38(7/8)、CD79a(6/9)、CD56(3/7)和PAX5(1/8)部分阳性,Ki67增殖指数高(70%~95%),EBER阳性8例。结论 PBL诊断困难,需结合临床表现、组织学特征、免疫表型和分子病理才能做出最终诊断,其预后差尚无有效的治疗方案。

Purpose To investigate the clinicopathological features,treatment,prognosis and differential diagnosis of plasmablastic lymphoma(PBL).Methods Collect clinical data of 9 cases of PBL,use HE and immunohistochemistry EnVision staining,detect EB virus using in situ hybridization,detect B lymphocyte gene rearrangement using PCR,analyze its clinical and pathological characteristics,and review relevant literature.Results Among the 9 patients,there were 7 males and 2 females,with a median age of 54 years.Two cases were found in the nasal cavity and sinuses,two cases in the lymph nodes,one cases in the gum,one cases in the tonsil,one cases in the skin,one in the colon and one in the anal canal.Among the 9 cases,4 were HIV positive.The skin lesions are plasma like cells,and the remaining areas are plasma like/immune like cells.Immunophenotype:CD138,MUM1 and C-myc were all positive.CD20,CD3 and CD5 were negative.Some of them expressed CD38(7/8),CD79a(6/9),CD56(3/7)and PAX5(1/8).Ki67 proliferation index was high(70%-95%).EBER in situ hybridization was positive in 8 cases.Conclusion The diagnosis of PBL is difficult and requires a combination of clinical manifestations,histological features,immunophenotype,and molecular pathology to make the final diagnosis.The prognosis of PBL is very poor and there are no effective treatment options.