转录因子MYC是一个重要的癌基因，在淋巴瘤发生中起着重要作用，但在弥漫性核心淋巴瘤（MCL）的临床结果中的重要性尚待确定。本研究对251名MCL患者的MYC蛋白表达进行了调查，并对部分患者进行了结构异常和mRNA的分析。14%（n = 35）的患者表现出高MYC蛋白表达，其中有超过20%的阳性细胞（MYChigh），其中仅检测到一起易位（translocation），而86%（n = 216）的患者表现出低MYC蛋白表达。在10名患者中检测到MYC拷贝数的低增益，但与MYC蛋白水平之间没有相关性。然而，MYC mRNA水平与MYC蛋白水平显著相关，其R2值为0.76。经过调整其他高风险特征后，MYChigh肿瘤患者的总生存期（OS）和无进展生存期（PFS）独立地降低（风险比（HR）= 2.03，95%置信区间（CI）1.2-3.4和HR = 2.2，95% CI 1.04-4.6）。MYChigh肿瘤的患者还倾向于具有额外的高风险特征，并且在诊断时年龄较大。有13名患者同时表达MYChigh和TP53 / p53改变，其进展风险（HR = 16.9，95% CI 7.4-38.3）和死亡风险（HR = 7.8，95% CI 4.4-14.1）显著增加，平均OS仅为0.9年。总之，我们表明，在诊断性MCL患者中有一个子群（14％）过表达MYC蛋白，并具有不良预后，但MYC重排很少见。同时高表达MYC和TP53 / p53变异的肿瘤指出MCL患者平均OS低于三年，预后极差。我们建议在MCL中评估MYC时，需要超越当前的高风险因素，以确定需要替代治疗的案例。

The transcription factor MYC is a well-described oncogene with an important role in lymphomagenesis, but its significance for clinical outcome in mantle cell lymphoma (MCL) remains to be determined. Here, we perform an investigation of the protein expression of MYC in a cohort of 251 MCL patients complemented with analyses of structural aberrations and mRNA, in a sub-cohort of patients. 14% (n=35) of patients showed high MYC protein expression with >20% positive cells (MYChigh), among which only one translocation was identified and 86% (n=216) of patients showed low MYC protein expression. Low copy number gains of MYC were detected in 10 patients, but with no correlation to MYC protein levels. However, MYC mRNA levels correlated significantly to MYC protein levels with a R2 value of 0.76. Patients with a MYChigh tumor had both an independent inferior overall survival (OS) and progression-free survival (PFS) (Hazard ratio (HR)=2.03, 95% Confidence interval (CI) 1.2-3.4 and HR=2.2, 95%CI 1.04-4.6, respectively) when adjusted for additional high-risk features. Patients with MYChigh tumors also tended to have additional high-risk features and to be older at diagnosis. A subgroup of 13 patients had concomitant MYChigh expression and TP53/p53 alterations and had a substantially increased risk of progression (HR=16.9, 95%CI 7.4-38.3) and death (HR=7.8, 95%CI 4.4-14.1) with an average OS of only 0.9 years. In summary, we show that a subset of diagnostic MCL patients (14%) overexpress MYC protein, and has a poor prognosis but that MYC rearrangements are rare. Tumors with concurrent MYC overexpression and TP53/p53 alterations pinpoint MCL patients with a dismal prognosis below three years of median OS. We propose that MYC needs to be assessed beyond the current high-risk factors in MCL to identify cases in need of alternative treatment.