肝血管内大B细胞性淋巴瘤2例临床病理观察

目的探讨肝血管内大B细胞性淋巴瘤的临床病理特征、诊断与鉴别诊断、治疗及预后。方法回顾性分析2例肝血管内大B细胞性淋巴瘤患者的临床资料、组织病理学形态和免疫组化结果。结果光镜下肝窦内和小血管内可见较多具有明显异型性的淋巴样细胞浸润,汇管区可见慢性炎细胞浸润,亦可见少许异型淋巴细胞样细胞,未见明确纤维化。免疫组化示CD20、PAX5弥漫(+),CD3散在少许(+),Ki-67阳性率为70%,AE1/AE3、CD117和CD56均(-);其中例1 CD5弥漫(+)。结论血管内大B细胞性淋巴瘤是一种具有高度侵袭性的结外弥漫性大B细胞性淋巴瘤的亚型,由于该病临床表现多样及不典型性,造成了部分患者的诊断困难,因此,掌握临床病理及免疫组化特征对该病的诊断和鉴别诊断具有重要意义。

Intravasculer large B cell lymphoma of liver: a clinicopathologic analysis of two cases

Objective To investigate the clinicopathological features,diagnosis,differential diagnosis,therapy and prognosis of intravasculer large B cell lymphoma.Methods Retrospective analysis of the clinical data was conducted in two cases of liver intravascular large B-cell lymphoma,and the pathological and immunohistochemical results were analyzed.Results Under light microscope,there were many atypical lymphocyte-like cells infiltrating in the hepatic sinusoids and small vessels,and some chronic inflammatory cells were visible in the portal area,but no clear fibrosis.Immunohistochemistry showed that CD20 and of PAX5 were diffusely positive and CD3 was scatteredly positive,the positive rate of Ki-67 was about 70%,AE1/AE3,CD117 and CD56 were all negative,and CD5 was diffusely positive in case 1 but negative in case 2.Conclusions Intravascular large B-cell lymphoma is a highly aggressive subtype of extranodal DLBCL.Clinical features of the disease are diverse and not typical,resulting in diagnostic difficulties and delay in diagnosis in some patients.Therefore,understanding the clinical pathological and immunohistochemical features of this disease,will play an important role in the diagnosis and the differential diagnosis of IVLBCL.