鼻腔 HIV 阴性浆母细胞性淋巴瘤1例报道并文献回顾

摘要

目的：探讨浆母细胞性淋巴瘤（PBL）的病因、诊断、治疗及预后。方法：对2014年收治的1例鼻腔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。结果：1例患者诊断明确，HIV 阴性。以间断右侧鼻出血发病，后发现鼻腔有肿瘤生长，骨质破坏向右侧额窦、筛窦、上颌窦浸润并伴有双侧淋巴结转移。行鼻侧切开肿瘤切除术＋双侧颈淋巴结清扫术，术后病理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、MUM -1阳性，CyD1部分阳性，Ki -67阳性90％。EBER 原位杂交部分阳性。LCA、CD79a、CD20、CD30、ALK、Bcl -2、Bcl -6、CD10、CD3、CK、EMA、PAX-5阴性，符合浆母细胞性淋巴瘤。术后给予化学药物治疗，现肿瘤复发正在随访中。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。%Objective:To investigate the origin,diagnosis,differential diagnosis,treatment,and prognosis of plas-mablastic lymphoma(PBL). Methods:To retrospectively analyze the clinical data of one patient with PBL. Results:The patient was a 55 - year - old man. He was explicitly diagnosed to be HIV negative. He presented enlarged tumor in the right nose with lymph node metastasis. The tumor displayed the morphology characteristics of diffused large cell lymphoma and the typical plasma cell phenotype. Immunohistochemical analysis revealed that the cells stained posi-tively for CD38,CD138,MUM - 1,CyD1. Nuclear proliferation rate as assessed by Ki - 67 staining was approximately 90% . And the cells stained poorly or even negatively for LCA,CD79a,CD20,CD30,ALK,Bcl - 2,Bcl - 6,CD10, CD3,CK,EMA,PAX - 5. Histochemistry in situ hybridization of EBER was positive. CHOP regime showed no satis-factory effect. Relapse appeared quickly after a brief remission. Conclusion:PBL is very rare,with malignancy and poor prognosis. All PBL patients present low sensitivity to chemotherapy drugs and exhibit short survival rate. There is no standard chemotherapy regimen for PBL.