目的　探讨儿童伴IRF4基因重排大B细胞淋巴瘤的临床及病理特点。方法　回顾分析3例儿童伴IRF4基因 重排的大B细胞淋巴瘤患儿的临床资料。结果　3例患儿均为男性，发病年龄5岁4月龄至7岁10月龄，发病部位分别为 扁桃体、回盲部及颈部淋巴结。 3例患儿手术后组织病理形态及免疫组织化学检查显示，肿瘤细胞呈结节样或弥漫性分布， 瘤细胞均表达CD20、MUM1、BCL-6和BCL-2， 2例表达CD10。FISH检测IRF4基因， 2例断裂阴性、 1例阳性。均诊断为 伴IRF4基因重排的大B细胞淋巴瘤。 3例患儿均按CCCG-NHL-2016方案治疗，其中2例按CCCG-NHL-2016方案R2组 执行， 1例按R3组执行。 2例患儿已经结束化疗1年余，随访至今无复发； 1例颈部淋巴结起病的R2组患儿仍在治疗中，目 前已获得完全缓解。结论　儿童伴IRF4基因重排的大B细胞淋巴瘤少见，行FISH检测IRF4基因有助诊断，可按CCCGNHL-2016方案治疗。

Objective　To explore the clinical and pathological features of large B-cell lymphoma with IRF4 gene rearrangement in children. Method　The clinical data of large B cell lymphoma with IRF4 gene rearrangement in 3 children were retrospective analyzed. Results　All the three cases were male and the age at onset was from 5 years and 4 months to 7 years and 10 months. The onset sites were tonsils, ileocecal, and cervical lymph nodes. The findings in histopathological morphology and immunohistochemistry of 3 children after surgery showed that the tumor cells were nodular or diffusely distributed. The tumor cells all expressed CD20, MUM1, BCL-6, and BCL-2, and they expressed CD10 in 2 cases. FISH was performed and showed fracture in IRF4 gene were negative in 2 cases and was positive in 1 case. All patients were diagnosed of large B-cell lymphoma with IRF4 gene rearrangement. All 3 cases were treated according to the CCCG-NHL-2016 regimen, 2 cases in group R2 and 1 case in group R3. Two patients had finished chemotherapy for more than one year, and no recurrence was found up to now. A child in R2 group with onset site of cervical lymph node was still under treatment and had achieved complete remission. Conclusion　Large B-cell lymphoma with IRF4 gene rearrangement in children is rare. FISH detection of IRF4 gene is helpful for diagnosis and its treatment can be according to the CCCG-NHL-2016 regimen.