原发性肝脏弥漫大B细胞淋巴瘤7例临床病理学观察

Clinicopathological features of 7 cases primary hepatic diffuse large B-cell lymphoma

目的 探讨肝脏原发性弥漫大B细胞淋巴瘤（primary hepatic diffuse large B-cell lymphoma,PHDLBL）的影像学、组织学及免疫表型,分析其临床病理特征、诊断、鉴别诊断、治疗及预后。方法 对7例肝脏PHDLBL进行光镜观察及免疫组化标记,采用原位分子杂交法检测淋巴瘤组织中EBV编码的小RNA（EBV-encoded small RNA,EBER）,并结合相关文献复习。结果 7例患者平均年龄67.5岁,均以右上腹部饱胀不适、消瘦伴贫血就诊。CT示病灶密度欠均匀,可见更低密度区,增强扫描病灶不均匀强化。肿瘤细胞弥漫性生长,瘤细胞体积较大,大小相对一致,多为圆形,胞质较丰富、淡染,核圆形或卵圆形,染色质粗,可见明显的核仁和泡状核,核分裂象易见,并可见星空现象。免疫表型：瘤细胞弥漫表达CD19、CD20、CD79a,CD3、CD43均阴性,Ki-67增殖指数75%~80%;3例EBER原位杂交检测呈阳性。结论 PHDLBL临床罕见,预后较差,肝活检及免疫组化标记有助于诊断,需与其他部位的淋巴瘤累及肝脏鉴别;及早手术和化疗有望获得较好疗效。

Purpose To study images, histopathological features, and immunotypes of primary hepatic diffuse large B-cell lymphoma （PHDLBL） , and to explore the clinicopathologie characteristics, diagnosis, differential diagnosis and treatment. Methods Seven cases of PHDLBL were studied with histological, and immunohistochemical techniques. In situ hybridization was employed to detect the EBV-encoded small RNA （EBER） in tumor cells. The relevant literatures were also reviewed. Results The average age of the seven patients was 67.5 years. Moreover, bulge of the upper fight abdomen and progressive athrepsia and anemia were observed in all seven patients. Computed tomography （CT） revealed the presence of multiple solid hypodense lesions. Further, CT also revealed an enhanced irregular focus. Histopathological analysis revealed the following characteristics ： heavy infiltration composed mainly of mediumsized round cells with a lightly stained cytoplasm, prominent nucleoli and vesicular nuclei, nuclear fission and visible sky star phenomena. The tumor ceils showed diffuse expression of CD19, CD20, and CD79a. The Ki-67 index were 75% - 80%. The tumor cells in three cases were positive for EBER. Conclusion PHDLBL is very rare and highly malignant lymphoma with poor prognosis . Live biopsy and immunohistochemical staining is helpful for diagnosing this tumor. It should be paid attention to differentiates with other parts of the lymphoma involving the liver. Early surgery and chemotherapy could obtain good curative effect.