High-Grade B-Cell Lymphoma (HGBL) with MYC and BCL2 and/or BCL6 Rearrangements Is Predominantly BCL6-Rearranged and BCL6-Expressing in Taiwan

Cancers

Shu-Min Hsieh¹  Cheng-Chih Tsai²  Yung-Cheng Su²  Oluwaseun Adebayo Bamodu²  Chi-Tai Yeh²  Yao-Yu Hsieh²  Tsu-Yi Chao²  Hui-Wen Liu²  Wei-Hong Cheng²  Huey-En Tzeng³  Huan-Tze Lin³  Yu-Mei Zheng⁴  Jacqueline Whang-Peng⁴  Chia-Lun Chang⁴  Chi-Long Chen⁵  Chia-Lang Fang⁵  Mei-Ling Liu⁶  Bo-Jung Chen⁶  Chii-Hong Lee⁶  Wei-Hwa Lee⁶  Wen-Chiuan Tsai⁷  Shiue-Wei Lai⁸  Ching-Liang Ho⁸  Yun Yen⁹ 

[1] Department of Clinical Pathology, Taipei Medical University-Shuang Ho Hospital, New Taipei City 235, Taiwan;Department of Internal Medicine, Division of Hematology and Oncology, Taipei Medical University-Shuang Ho Hospital, New Taipei City 235, Taiwan;Department of Medicine, Division of Hematology and Oncology, Taipei Medical University Hospital, Taipei City 110, Taiwan;Department of Medicine, Division of Hematology and Oncology, Taipei Medical University-Wan-Fang Hospital, Taipei City 116, Taiwan;Department of Pathology, Taipei Medical University Hospital, Taipei City 110, Taiwan;Department of Pathology, Taipei Medical University-Shuang Ho Hospital, New Taipei City 235, Taiwan;Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei City 114, Taiwan;Division of Hematology-Oncology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei City 114, Taiwan;Program for Cancer Molecular Biology and Drug Discovery, College of Medical Science and Technology, Taipei Medical University, Taipei City 110, Taiwan;

关键词: MYC;    BCL2;    BCL6;    HGBL;    DLBCL;    double-hit lymphoma;   

DOI  :  10.3390/cancers13071620

来源: DOAJ

【 摘 要 】

This study investigated the epidemiological and clinical peculiarities of BCL2 and BCL6 rearrangement in patients with high grade B-cell lymphoma (HGBL) from Taiwan, compared with data from Western countries. Two hundred and eighty-two DLBCL cases from Taipei Medical University-affiliated hospitals (n = 179) and Tri-Service General Hospital (n = 103) were enrolled for this study. From the 282, 47 (16.7%) had MYC translocation; 24 of these harbored concurrent BCL2 and/or BCL6 translocation (double-hit, DH or triple-hit, TH). Twelve DH-HGBL cases had simultaneous MYC and BCL6 translocations, 8 harbored MYC and BCL2 rearrangement, while the remaining 4 patients exhibited TH. Together, 66.7% of DH/TH-HGBL patients were BCL6 rearrangement positive. Among these BCL6-rearranged DH/TH-HGBL patients, only 6 (37.5%) overexpressed MYC and BCL6 proteins simultaneously, indicating that MYC-BCL6 co-overexpression may not be plausible surrogate biomarker for screening BCL6-rearranged DH-HGBL. By the end of year 5, all patients with TH-HGBL, BCL2 DH-HGBL and all but one BCL6 DH-HGBL cases had expired or were lost to follow-up. Progression-free survival (PFS) was longer for the non-DH/TH-HGBL group compared with the DH/TH-HGBL group. While the patients with BCL2 DH-HGBL were lost to follow-up by day 800, their remaining TH-HGBL and BCL6 DH-HGBL peers exhibited very poor PFS, regardless of age strata. More so, patients with BCL6 rearrangement were 5.5-fold more likely associated with extranodal involvement compared with their BCL2-rearranged peers. Moreover,~60.0% of the BCL6-rearranged DH-HGBL cases were non-GCB, suggesting that including screening for BCL6 rearrangement in patients with the non-GCB phenotype may aid medical decision-making and therapeutic strategy. Contrary to contemporary data from western countries, 2 in every 3 patients with DH/TH-HGBL in Taiwan harbor BCL6 rearrangement. Consistent with present findings, we recommend mandatory screening for BCL6 rearrangement in patients with aggressive HGBL in Taiwan.

【 授权许可】

Unknown