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双重ALK和MYC重排导致间变性大细胞淋巴瘤的侵袭性变体。

Dual ALK and MYC rearrangements leading to an aggressive variant of anaplastic large cell lymphoma.

作者信息

Liang Xiayuan, Branchford Brian, Greffe Brian, McGavran Loris, Carstens Billie, Meltesen Lynne, Albano Edith A, Quinones Ralph, Cook Bruce, Graham Douglas K

机构信息

Department of Pathology, Children's Hospital Colorado, Aurora, CO 80045, USA.

出版信息

J Pediatr Hematol Oncol. 2013 Jul;35(5):e209-13. doi: 10.1097/MPH.0b013e3182815046.

Abstract

Anaplastic lymphoma kinase (ALK) and MYC are oncogenes often dysregulated in pediatric lymphomas. NPM-ALK/t(2;5)(p23;q35) is a genetic hallmark of ALK anaplastic large cell lymphoma (ALCL). MYC gene translocations are frequently detected in high-grade B-cell lymphomas. ALKALCL cases with concurrent MYC translocation are exceedingly rare and are more aggressive and chemoresistent compared with other ALKALCL. We report a patient who presented with ALKALCL possessing coexistent MYC rearrangement, massive tumor dissemination, and early widespread relapse. This case underscores the importance of recognition of close correlation between dual ALK and MYC rearrangements and the characteristic clinical features in this unusual ALCL variant.

摘要

间变性淋巴瘤激酶(ALK)和MYC是儿科淋巴瘤中经常失调的致癌基因。NPM-ALK/t(2;5)(p23;q35)是ALK间变性大细胞淋巴瘤(ALCL)的基因标志。MYC基因易位在高级别B细胞淋巴瘤中经常被检测到。同时发生MYC易位的ALK-ALCL病例极为罕见,与其他ALK-ALCL相比,侵袭性更强且对化疗耐药。我们报告了一名患有ALK-ALCL且伴有MYC重排、大量肿瘤播散和早期广泛复发的患者。该病例强调了认识双重ALK和MYC重排之间的密切相关性以及这种不寻常的ALCL变异型的特征性临床特征的重要性。

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