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

This study investigated the epidemiological and clinical peculiarities of and 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 ( = 179) and Tri-Service General Hospital ( = 103) were enrolled for this study. From the 282, 47 (16.7%) had translocation; 24 of these harbored concurrent and/or translocation (double-hit, DH or triple-hit, TH). Twelve DH-HGBL cases had simultaneous and translocations, 8 harbored and rearrangement, while the remaining 4 patients exhibited TH. Together, 66.7% of DH/TH-HGBL patients were rearrangement positive. Among these -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 -rearranged DH-HGBL. By the end of year 5, all patients with TH-HGBL, DH-HGBL and all but one 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 DH-HGBL were lost to follow-up by day 800, their remaining TH-HGBL and DH-HGBL peers exhibited very poor PFS, regardless of age strata. More so, patients with rearrangement were 5.5-fold more likely associated with extranodal involvement compared with their BCL2-rearranged peers. Moreover, ~60.0% of the -rearranged DH-HGBL cases were non-GCB, suggesting that including screening for 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 rearrangement. Consistent with present findings, we recommend mandatory screening for rearrangement in patients with aggressive HGBL in Taiwan.