临床儿科杂志 ›› 2020, Vol. 38 ›› Issue (9): 655-.doi: 10.3969/j.issn.1000-3606.2020.09.004

• 血液肿瘤疾病专栏 • 上一篇    

儿童伴IRF4 基因重排大B 细胞淋巴瘤临床病理分析

吴崇军, 黄慧, 熊婷, 徐忠金   

  1. 江西省儿童医院 南昌大学附属儿童医院(江西南昌 330000)
  • 发布日期:2020-09-17
  • 通讯作者: 徐忠金 电子信箱:xuzhongjin691129@sina.com
  • 基金资助:
    江西省卫生计生委科技计划项目(No.20185443);江西省重点研发计划项目 (No.20181BBG78011);江西省卫生计生委 科技计划项目(No.20185440)

Clinicopathological analysis of large B-cell lymphoma with IRF4 gene rearrangement in children

WU Chongjun, HUANG Hui, XIONG Ting, XU Zhongjin   

  1. Jiangxi Provincial Children's Hospital, The Affiliated Children's Hospital of Nanchang University, Nanchang 330000, Jiangxi, China
  • Published:2020-09-17

摘要: 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。

关键词: 伴IRF4基因重排的大B细胞淋巴瘤; 病理; 治疗; 儿童

Abstract: 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.

Key words:  large B-cell lymphoma with IRF4 gene rearrangement; pathology; treatment; child