TY - JOUR
T1 - MYC rearrangement but not extra MYC copies is an independent prognostic factor in patients with mantle cell lymphoma
AU - Wang, Lifu
AU - Tang, Guilin
AU - Medeiros, L. Jeffrey
AU - Xu, Jie
AU - Huang, Wenting
AU - Yin, C. Cameron
AU - Wang, Michael
AU - Jain, Preetesh
AU - Lin, Pei
AU - Li, Shaoying
N1 - Publisher Copyright:
© 2021 Ferrata Storti Foundation
PY - 2021/5
Y1 - 2021/5
N2 - 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 MYC-NL. 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 intensive 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, Ann Arbor stage IV 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.
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 MYC-NL. 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 intensive 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, Ann Arbor stage IV 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.
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U2 - 10.3324/haematol.2019.243071
DO - 10.3324/haematol.2019.243071
M3 - Article
C2 - 32273477
AN - SCOPUS:85105437717
SN - 0390-6078
VL - 106
SP - 1381
EP - 1389
JO - Haematologica
JF - Haematologica
IS - 5
ER -