High-grade b-cell lymphoma (Hgbl) with myc and bcl2 and/or bcl6 rearrangements is predominantly bcl6-rearranged and bcl6-expressing in Taiwan

Cheng Chih Tsai, Yung Cheng Su, Oluwaseun Adebayo Bamodu, Bo Jung Chen, Wen Chiuan Tsai, Wei Hong Cheng, Chii Hong Lee, Shu Min Hsieh, Mei Ling Liu, Chia Lang Fang, Huan Tze Lin, Chi Long Chen, Chi Tai Yeh, Wei Hwa Lee, Ching Liang Ho, Shiue Wei Lai, Huey En Tzeng, Yao Yu Hsieh, Chia Lun Chang, Yu Mei ZhengHui Wen Liu, Yun Yen, Jacqueline Whang-Peng, Tsu Yi Chao

Research output: Contribution to journalArticlepeer-review

Abstract

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/THHGBL 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 decisionmaking 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.

Original languageEnglish
Article number1620
JournalCancers
Volume13
Issue number7
DOIs
Publication statusPublished - Apr 1 2021

Keywords

  • BCL2
  • BCL6
  • DLBCL
  • Double-hit lymphoma
  • Gene rearrangement
  • HGBL
  • MYC
  • Non-GCB
  • Non-Hodgkin’s lymphoma

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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