血液肿瘤疾病专栏

儿童伴IRF4 基因重排大B 细胞淋巴瘤临床病理分析

  • WU Chongjun ,
  • HUANG Hui ,
  • XIONG Ting ,
  • et al
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  • 江西省儿童医院 南昌大学附属儿童医院(江西南昌 330000)

网络出版日期: 2020-09-17

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

  • 吴崇军,黄慧,熊婷,等
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  • Jiangxi Provincial Children's Hospital, The Affiliated Children's Hospital of Nanchang University, Nanchang 330000, Jiangxi, China

Online 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。

本文引用格式

WU Chongjun , HUANG Hui , XIONG Ting , et al . 儿童伴IRF4 基因重排大B 细胞淋巴瘤临床病理分析[J]. 临床儿科杂志, 2020 , 38(9) : 655 . DOI: 10.3969/j.issn.1000-3606.2020.09.004

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.
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