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中华危重症医学杂志(电子版) ›› 2022, Vol. 15 ›› Issue (06) : 515 -519. doi: 10.3877/cma.j.issn.1674-6880.2022.06.017

病例报告

原发心脏伴11号染色体长臂异常的高级别B细胞淋巴瘤一例并文献复习
谢洪琼1, 叶杏浓1, 杨春梅1, 张翔1, 杨洁1, 俞文娟1,()   
  1. 1. 310003 杭州,浙江大学医学院附属第一医院血液科
  • 收稿日期:2022-10-30 出版日期:2022-12-31
  • 通信作者: 俞文娟
  • 基金资助:
    浙江大学2021年省教育厅一般科研项目(Y202147531)
  • Received:2022-10-30 Published:2022-12-31
引用本文:

谢洪琼, 叶杏浓, 杨春梅, 张翔, 杨洁, 俞文娟. 原发心脏伴11号染色体长臂异常的高级别B细胞淋巴瘤一例并文献复习[J]. 中华危重症医学杂志(电子版), 2022, 15(06): 515-519.

伴11号染色体长臂异常的高级别B细胞淋巴瘤(high-grade B-cell lymphoma with 11q aberrations,HGBCL-11q)在世界卫生组织(World Health Organization,WHO)2022年6月发布的第五版《淋巴造血系统肿瘤分类及其诊断标准》中被列为一个新的淋巴瘤亚型[1]。HGBCL-11q发病率比较低,占所有淋巴瘤的0.19%[2]。国内外HGBCL-11q的病例多见于儿童和青少年;男性多于女性,男女比为6.63 ∶ 1;发病部位以淋巴结多见,且以头颈部、鼻咽部和腹部淋巴结侵犯最常见[2-4],结外病变少见,原发心脏的HGBCL-11q更为罕见。目前HGBCL-11q尚无标准治疗方案,大部分患者依据伯基特淋巴瘤(Burkitt lymphoma,BL)的标准方案进行化学治疗[5],但预后比BL好。本文报道浙江大学医学院附属第一医院收治的1例原发心脏的HGBCL-11q患者,对其临床资料进行回顾性分析,并进行相关文献复习。

图1 原发心脏的HGBCL-11q患者治疗前和治疗中期影像图注:HGBCL-11q.伴11号染色体长臂异常的高级别B细胞淋巴瘤;PET-CT.正电子发射计算机断层显像;SUVmax.最大标准摄取值;a图为治疗前超声心动图显示右心房巨大占位,大小10.3 cm × 7.2 cm × 8.3 cm,影响上腔静脉入口;b、c图为治疗前PET-CT显示右心房及右心室内巨大软组织密度肿块影,左心房局部受累,侵入心包腔,放射性摄取增高,SUVmax约27.7;d图为治疗4个疗程后超声心动图显示心包腔内低回声团块,大小3.6 cm × 1.3 cm,形态不规则;e、f图为治疗4个疗程后PET-CT显示右心旁心包内及纵隔血管间隙内不规则条状软组织密度影,SUVmax约3.7
图2 原发心脏的HGBCL-11q患者手术标本病理特征注:HGBCL-11q.伴11号染色体长臂异常的高级别B细胞淋巴瘤;HE.苏木素-伊红;BCL-6. B细胞淋巴瘤基因6;FISH.荧光染色体原位杂交;a图示中等大小淋巴样细胞弥漫片状镶嵌样排列,染色质细腻,核仁不明显,核分裂像及凋亡易见(HE染色 × 40);b图示CD20(+);c图示CD3(-);d图示CD10(+);e图示BCL-6(+);f图示BCL-2(-);g图示c-MYC(+,40%);h图示Ki-67(+,>90%);i图采用11q23.3/11q24.3探针,结果显示11q23.3位点(红信号)发生扩增,11q24.3位点(绿信号)发生缺失(FISH法 × 1 000)
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