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目的探讨脾弥漫性红髓小B细胞淋巴瘤(SDRPSBL)的临床病理学特征,诊断及鉴别诊断。方法收集2014-03—2016-08北京大学人民医院病理科诊断的SDRPSBL 2例,按WHO(2008)淋巴造血组织肿瘤分类和WHO(2016)更新内容,应用光镜观察、免疫组化染色,结合临床相关检查进行临床病理学分析,并复习相关文献。结果例1:女性,51岁,表现为贫血,淋巴细胞减低。例2:男性,40岁,淋巴细胞升高,LDH升高。2例均表现为白细胞升高,脾肿大,行脾切除术。镜下:脾红髓、白髓结构不清楚,可见弥漫浸润的淋巴样细胞,细胞小~中等大小,沿窦隙及窦索浸润,可见广泛出血,血湖形成。免疫组化染色:CD20和PAX-5(+),CD3、CD5、CD23(未见FDC网)、Cyclin D1、CD38和CD25均(-),Ki-67(5%,10%)。例1失访,例2一般状况良好。结论 SDRPSBL是一种独特的病种,病例少见,以小~中等大淋巴细胞弥漫性浸润脾红髓为主要特征,形态学及免疫表型不同于其他脾B细胞淋巴瘤。
Objective To investigate the clinicopathological features, diagnosis and differential diagnosis of splenic diffuse red pulp small B cell lymphoma (SDRPSBL). Methods Two cases of SDRPSBL diagnosed by Department of Pathology, Peking University People’s Hospital from March 2014 to June 2016 were collected. According to the WHO (2008) classification of lymphoid hematopoietic tumors and WHO (2016), light microscopy, immunohistochemical staining, Clinic-related examination of clinical pathology, and review the relevant literature. Results Example 1: Female, 51 years old, manifested as anemia, lymphopenia. Example 2: Male, 40 years old, with elevated lymphocytes and elevated LDH. 2 cases showed leukocytosis, splenomegaly, splenectomy. Microscope: spleen red pulp, white pulp structure is not clear, we can see diffuse infiltration of lymphoid cells, small to medium size, infiltration along the sinus gap and sinus, showing extensive bleeding, the formation of blood lake. Immunohistochemical staining: CD20 and PAX-5 (+), CD3, CD5, CD23 (no FDC net), Cyclin D1, CD38 and CD25 both (-) and Ki-67 (5%, 10%). Example 1 lost, Example 2 generally good condition. Conclusion SDRPSBL is a unique disease with rare cases. Diffuse infiltration of spleen-red marrow into small-medium-sized lymphocytes is the main characteristic. The morphology and immunophenotype of SDRPSBL are different from other splenic B-cell lymphoma.