MYC single-hit large B-cell lymphoma: clinicopathologic difference from MYC-negative large B-cell lymphoma and MYC double-hit/triple-hit lymphoma

• Published in: Human pathology Vol. 113; pp. 9 - 19
• Main Authors: Cho, Yoon Ah, Hyeon, Jiyeon, Lee, Hyunwoo, Cho, Junhun, Kim, Seok-Jin, Kim, Won Seog, Ko, Young-Hyeh
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-07-2021; Elsevier Limited
• Subjects: Biomarkers, Tumor - genetics; Cell cycle; Disease Progression; Double hit; Female; Gene Rearrangement; Genetic Predisposition to Disease; Genotype & phenotype; Humans; Hybridization; Immunohistochemistry; In Situ Hybridization, Fluorescence; Large B-cell lymphoma; Lymphoma; Lymphoma, Large B-Cell, Diffuse - genetics; Lymphoma, Large B-Cell, Diffuse - mortality; Lymphoma, Large B-Cell, Diffuse - pathology; Lymphoma, Large B-Cell, Diffuse - therapy; Male; Medical prognosis; Middle Aged; Morphology; MYC; Neoplasm Grading; Phenotype; Prognosis; Progression-Free Survival; Proto-Oncogene Proteins c-bcl-2 - genetics; Proto-Oncogene Proteins c-bcl-6 - genetics; Proto-Oncogene Proteins c-myc - genetics; Single hit; Time Factors; Translocation; United States > US; Translocation; Double hit; Prognosis; Large B-cell lymphoma; Single hit; MYC
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2021.03.006

MYC-rearranged large B-cell lymphoma with BCL2 and/or BCL6 rearrangement, double-hit (DH) or triple-hit (TH) lymphoma, is associated with poor survival after standard treatment. To investigate the clinical impact of single-hit (SH) MYC rearrangement, we analyzed 241 cases of diffuse large B-cell lymphoma (DLBCL) for MYC, BCL2, and BCL6 rearrangement by fluorescence in situ hybridization. Fifty-five of 241 (22.8%) cases showed MYC rearrangements. Twenty-three cases were diagnosed as DLBCL; 18 as high-grade B-cell lymphoma (HGBCL)-DH; 3 as HGBCL-TH; and 11 as HGBCL, not otherwise specified. Both DH and TH lymphomas showed high-grade morphology (P = 0.002), higher stage (P = 0.022), and more frequent germinal center B-cell-like phenotype (P = 0.008). SH lymphomas displayed high-grade morphology (P = 0.002) but were not different from MYC-negative lymphomas in cell of origin, clinical stage, international prognostic index (IPI), or extranodal involvement. Patients with DH/TH lymphomas had worse overall survival (OS) (P = 0.016) and progression-free survival (PFS) (P < 0.001), while OS and PFS of SH lymphomas were not different from those of MYC-negative lymphomas. There was no survival difference between cases of BCL2 and BCL6 rearrangements. Poorer prognostic factors included higher ECOG class, higher IPI, and DH or TH translocation for OS, and higher IPI and DH or TH translocation for PFS. Higher IPI was an independent prognostic factor for OS and PFS. In conclusion, large B-cell lymphomas with single MYC rearrangement showed high-grade morphology but were otherwise not different from MYC-negative lymphomas.