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高级别B细胞淋巴瘤合并MYC/BCL2/BLC6重排

High grade B-cell lymphoma with MYC and/ or BCL2 and/or BCL6 rearrangement

ORPHA:480541疾病

定义 英文原文(暂无中文)

A rare aggressive B-cell non-Hodgkin lymphoma characterized by a rearrangement in MYC and BCL2 and/or BCL6 (so-called double-hit or triple-hit lymphoma). The category includes double-hit cases with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, blastoid cases with a double-hit, and cases with a DLBCL, not otherwise specified, morphology with a double-hit. It refers only to de novo cases, not to lymphomas with a history of pre-existing or coexistent indolent lymphoma. Patients typically present with widespread disease, including involvement of lymph nodes, bone marrow, and central nervous system.

基本事实

遗传方式
不适用

相关基因 3

基因名称关联类型
BCL2BCL2 apoptosis regulatorPart of a fusion gene in
BCL6BCL6 transcription repressorPart of a fusion gene in
MYCMYC proto-oncogene, bHLH transcription factorPart of a fusion gene in

外部标识与链接

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本页数据来源

  • 疾病定义、同义词、基因、表型、流行病学:Orphanet(CC BY 4.0)
  • 中文病名:Orphanet 中文包,冻结于 2020-06-01
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)