Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KS 66160, USA.
Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KS 66160, USA.
Hum Pathol. 2019 Apr;86:21-31. doi: 10.1016/j.humpath.2018.11.015. Epub 2018 Nov 26.
p53 expression and MYC extra copies (MYC-EC) have been reported to serve as independent adverse prognostic markers in patients with diffuse large B-cell lymphoma (DLBCL). However, the impact of p53 expression in MYC-EC lymphomas has not been delineated. Conversely, CD99 expression has been shown to have a positive impact on survival in patients with germinal center-type DLBCL, yet nothing is reported about the impact of CD99 expression and MYC status. This is the first study to evaluate p53 expression in MYC-EC lymphomas and CD99 expression in relation to MYC status. We identified 122 patients diagnosed as having large B-cell lymphoma (44, MYC-negative; 29, MYC-EC; 23, MYC rearrangement; 22, MYC and BCL2 rearrangements; 4, MYC, BCL2, and BCL6 rearrangements). p53 expression significantly correlated with DLBCL with abnormal MYC status (MYC-EC, MYC rearrangement, and MYC overexpression), but adverse p53 prognostic effect was only seen with MYC-rearranged lymphoma. CD99 expression was significantly associated with MYC-negative DLBCL and had better prognostic impact on lymphoma-specific survival (LSS), but not on relapse-free survival and overall survival. Overall, patients with MYC-EC lymphoma had significantly worse relapse-free survival and LSS than did patients with MYC-negative lymphoma, yet better overall survival and LSS than did the patients with MYC-rearranged lymphoma. Thus, patients with MYC-EC lymphomas had prognostic features that were intermediate between MYC-negative and MYC-rearranged lymphomas. Lastly, high-intensity chemotherapy (either dose-adjusted rituximab and etoposide-prednisone-vincristine-cyclophosphamide-doxorubicin or rituximab and hyperfractionated cyclophosphamide-vincristine-doxorubicin-dexamethasone treatment) did not improve survival in patients with MYC-EC and MYC-rearranged lymphoma when compared with rituximab-cyclophosphamide-hydroxydaunomycin-vincristine-prednisone therapy.
p53 表达和 MYC 额外拷贝（MYC-EC）已被报道为弥漫性大 B 细胞淋巴瘤（DLBCL）患者的独立不良预后标志物。然而，p53 表达在 MYC-EC 淋巴瘤中的影响尚未阐明。相反，CD99 表达已被证明对生发中心型 DLBCL 患者的生存有积极影响，但关于 CD99 表达和 MYC 状态的影响尚无报道。这是第一项评估 MYC-EC 淋巴瘤中 p53 表达和 MYC 状态与 CD99 表达的关系的研究。我们鉴定了 122 名被诊断为患有大 B 细胞淋巴瘤的患者（44 名，MYC 阴性；29 名，MYC-EC；23 名，MYC 重排；22 名，MYC 和 BCL2 重排；4 名，MYC、BCL2 和 BCL6 重排）。p53 表达与异常 MYC 状态的 DLBCL（MYC-EC、MYC 重排和 MYC 过表达）显著相关，但不良的 p53 预后作用仅见于 MYC 重排的淋巴瘤。CD99 表达与 MYC 阴性 DLBCL 显著相关，对淋巴瘤特异性生存（LSS）有更好的预后影响，但对无复发生存和总生存无影响。总体而言，与 MYC 阴性淋巴瘤患者相比，MYC-EC 淋巴瘤患者的无复发生存和 LSS 显著更差，但总生存和 LSS 更好，与 MYC 重排淋巴瘤患者相比。因此，MYC-EC 淋巴瘤患者的预后特征介于 MYC 阴性和 MYC 重排淋巴瘤患者之间。最后，高强度化疗（剂量调整的利妥昔单抗和依托泊苷-泼尼松-长春新碱-环磷酰胺-阿霉素或利妥昔单抗和超分割环磷酰胺-长春新碱-阿霉素-地塞米松治疗）与利妥昔单抗-环磷酰胺-阿霉素-长春新碱-泼尼松治疗相比，并未改善 MYC-EC 和 MYC 重排淋巴瘤患者的生存。
