Yang C R, Hu Y C, Zhou X G, Zhang D
Department of Pathology, the First College of Clinical Medical Science, China Three Gorges University, Yichang 443003, China; Institute of Pathology, China Three Gorges University, Yichang 443003, China.
Department of Pathology, Beijing Friendship Hospital of the Capital University of Medicine Science, Beijing 100050, China.
Zhonghua Bing Li Xue Za Zhi. 2019 Feb 8;48(2):132-136. doi: 10.3760/cma.j.issn.0529-5807.2019.02.011.
To investigate the clinicopathologic features of follicular lymphoma (FL) in children. One female and one male patients with FL diagnosed in the First College of Clinical Medical Science, China Three Gorges University and Beijing Friendship Hospital of the Capital University of Medical Science in February 2016 and June 2015 were studied by HE immunohistochemistry, EBER in situ hybridization, IgH and IgK gene rearrangement analysis and IRF4 fusion gene detection. The two patients' age were 6.3 and 12 years, respectively. The lesions involved head and neck lymph nodes with duration of more than 2 months. Histopathologically, the lesions consisted of nodular proliferation of lymphoid follicles with diffuse distribution of large cells. Starry sky phenomenon was seen in one of the two cases. Immunohistochemistry showed that one case was positive for bcl-2 and MUM1, but negative for bcl-6 and CD10. Ki-67 index was>50% and oligoclonal IgK rearrangement was observed. The second case showed positivity for bcl-6, and CD10 but negative for bcl-2. Ki-67 index was>50% and clonal IgH FR1-JH and IgH FR2-JH rearrangements were detected. Both cases showed no evidence of IRF4 gene fusion. Childhood FL is a rare B-cell lymphoma with characteristic features and high-grade histomorphology. However, its immunophenotype and molecular genetic characteristics are divergent.
探讨儿童滤泡性淋巴瘤（FL）的临床病理特征。对2016年2月在中国三峡大学第一临床医学院以及2015年6月在首都医科大学附属北京友谊医院确诊为FL的1例女性和1例男性患者进行了HE免疫组化、EBER原位杂交、IgH和IgK基因重排分析以及IRF4融合基因检测。两名患者年龄分别为6.3岁和12岁。病变累及头颈部淋巴结，病程超过2个月。组织病理学上，病变由淋巴滤泡结节状增生伴大细胞弥漫分布组成。两例中的一例可见星空现象。免疫组化显示，1例bcl-2和MUM1阳性，但bcl-6和CD10阴性。Ki-67指数>50%，观察到寡克隆IgK重排。第二例bcl-6和CD10阳性，但bcl-2阴性。Ki-67指数>50%，检测到克隆性IgH FR1-JH和IgH FR2-JH重排。两例均未发现IRF4基因融合证据。儿童FL是一种罕见的B细胞淋巴瘤，具有特征性表现和高级别组织形态学。然而，其免疫表型和分子遗传学特征存在差异。
