Cho Yoon Ah, Hyeon Jiyeon, Lee Hyunwoo, Cho Junhun, Kim Seok-Jin, Kim Won Seog, Ko Young-Hyeh
Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Republic of Korea; Department of Pathology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang 14068, Republic of Korea.
Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Republic of Korea.
Hum Pathol. 2021 Jul;113:9-19. doi: 10.1016/j.humpath.2021.03.006. Epub 2021 Mar 23.
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.
伴有BCL2和/或BCL6重排的MYC重排大B细胞淋巴瘤、双打击（DH）或三打击（TH）淋巴瘤，在接受标准治疗后生存率较低。为了研究单打击（SH）MYC重排的临床影响，我们通过荧光原位杂交分析了241例弥漫性大B细胞淋巴瘤（DLBCL）的MYC、BCL2和BCL6重排情况。241例（22.8%）病例显示有MYC重排。23例被诊断为DLBCL；18例为高级别B细胞淋巴瘤（HGBCL）-DH；3例为HGBCL-TH；11例为未另行特指的HGBCL。DH和TH淋巴瘤均显示出高级别形态（P = 0.002）、更高分期（P = 0.022）以及更频繁的生发中心B细胞样表型（P = 0.008）。SH淋巴瘤表现出高级别形态（P = 0.002），但在起源细胞、临床分期、国际预后指数（IPI）或结外受累方面与MYC阴性淋巴瘤无差异。DH/TH淋巴瘤患者的总生存期（OS）较差（P = 0.016），无进展生存期（PFS）也较差（P < 0.001），而SH淋巴瘤的OS和PFS与MYC阴性淋巴瘤无差异。BCL2和BCL6重排病例之间的生存率无差异。较差的预后因素包括较高的ECOG分级、较高的IPI以及OS的DH或TH易位，和PFS的较高IPI以及DH或TH易位。较高的IPI是OS和PFS的独立预后因素。总之，伴有单一MYC重排的大B细胞淋巴瘤表现出高级别形态，但在其他方面与MYC阴性淋巴瘤无差异。
