目的 探讨儿童滤泡性淋巴瘤(FL)的临床病理学特征,以提高对该类疾病的认识.方法 收集三峡大学第一临床医学院2016年2月(例1)及首都医科大学附属北京友谊医院2015年6月(例2)诊断的儿童FL,采用HE染色、免疫组织化学、显色原位杂交、聚合酶链反应(PCR)及荧光原位杂交(FISH)技术,观察其组织学特征、免疫表型、EB病毒编码的小RNA(EBER)、B细胞克隆分析及IRF4融合基因检测,随访患者,并复习相关文献.结果 患儿男1例,女1例,年龄6岁3个月和12岁,发病部位均为头颈部淋巴结,起病时间均为2个月余且无其他病灶,组织病理学上均表现为膨胀的大滤泡,呈大结节分布,结节内细胞弥漫分布,细胞较大,类似中心母细胞,例2可见星空现象.免疫组织化学例1显示结节内bcl-2、MUM1阳性,bcl-6弱阳性,CD10阴性,Ki-67阳性指数>50%;例2显示结节内bcl-6、CD10阳性,bcl-2阴性,Ki-67阳性指数>50%.例1遗传学示IgK VK-JK呈寡克隆性重排.例2遗传学示IgH FR1-JH呈单克隆性重排,IgH FR2-JH呈单克隆性重排.2例IRF4融合基因检测显示均无IRF4断裂.EBER均阴性.结论 儿童FL是一种罕见的B细胞肿瘤,其发病部位具特征性,显示高级别病变或强的侵袭性形态学特征,但其免疫表型及分子遗传学特征又有不同,亟需我们提高对该类疾病的认识.
Objective To investigate the clinicopathologic features of follicular lymphoma (FL) in children.Methods 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.Results 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.Stan-y 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.Conclusions Childhood FL is a rare B-cell lymphoma with characteristic features and high-grade histomorphology.However,its immunophenotype and molecular genetic characteristics are divergent.