리툭시맙 치료를 받은 더블 익스프레서 미만성 큰 B 세포 림프종에서 스테리-스카이 패턴이 갖는 임상병리학적 및 분자유전학적 특징

Abstract

Double expressor lymphoma (DEL) is a subset of diffuse large B-cell lymphoma (DLBCL) characterized by the co-expression of MYC and BCL2 proteins with a poor prognosis. However, there are no standard criteria for evaluating the morphologic features of DEL. We aimed to analyze the prognostic value of the starry-sky pattern (SSP) and its correlation with clinicopathologic and genetic features in 158 DEL cases. The SSP was significantly associated with aggressive parameters, including c-MYC overexpression, CD5 expression, higher International prognostic index (IPI), and higher age-adjusted IPI. In the univariate survival analyses, the presence of SSP was associated with unfavorable progression-free survival (PFS) (p = 0.030), and tended towards an adverse overall survival (OS) (p = 0.065). However, when c-MYC was overexpressed, SSP was significantly correlated with inferior OS (p = 0.025). In the multivariate survival analysis, SSP was also associated with poor PFS (p = 0.049). Additionally, next-generation sequencing data revealed DEL with SSP was significantly associated with the KMT2D mutation and had different genetic mutation profiles from DEL without SSP. In conclusion, SSP may represent morphologic characteristics of aggressiveness in DEL.