TY - JOUR AU - Lifu Wang, AU - Guilin Tang, AU - L. Jeffrey Medeiros, AU - Jie Xu, AU - Wenting Huang, AU - C. Cameron Yin, AU - Michael Wang, AU - Preetesh Jain, AU - Pei Lin, AU - Shaoying Li, PY - 2021/04/30 Y2 - 2024/03/28 TI - <i>MYC</i> rearrangement but not extra <i>MYC</i> copies is an independent prognostic factor in patients with mantle cell lymphoma JF - Haematologica JA - haematol VL - 106 IS - 5 SE - Articles DO - 10.3324/haematol.2019.243071 UR - https://haematologica.org/article/view/9709 SP - 1381-1389 AB - Mantle cell lymphoma (MCL) with MYC rearrangement (MYC-R) is rare and little is known about the importance of MYC extra copies (EC) in the absence of MYC-R in MCL patients. This study includes 88 MCL patients with MYC tested by fluorescence in situ hybridization and/or conventional cytogenetics, including 27 with MYC-R, 21 with MYC-EC, and 40 with normal (NL) MYC. MCL patients with MYC-R more often had blastoid/pleomorphic morphology; a higher frequency of CD10, MYC, and simultaneous MYC and BCL2 expression; a higher level of MYC; and a higher Ki67 proliferation rate (p<0.05) than those without MYC-R. Although patients with MYC-R more frequently received aggressive chemotherapy (p=0.001), their overall survival (OS) was significantly shorter than those without MYC-R. Compared with patients with MYC/BCL2 double hit lymphoma (DHL), patients with MYC-R MCL had a similar OS but more commonly had bone marrow involvement, stage 4 disease, and a different immunophenotype. MCL patients with MYC-EC showed an OS intermediate between those with MYC-R and MYC-NL, either all or only blastoid/pleomorphic MCL patients included. Multivariate analysis showed that MYC-R, but not MYC-EC, had an independent and negative impact on OS. In conclusion, MYC-R but not MYC-EC showed a higher MYC expression and is an adverse prognostic factor for MCL patients. Although the OS of MCL patients with MYC-R is similar to that of MYC/BCL2 DHL patients, these groups have different clinicopathologic features supporting the retention of MCL with MYC-R in the category of MCL, as recommended in the revised World Health Organization classification. ER -