Chen Rongrong, Zhou De, Wang Lulu, Zhu Lixia, Ye Xiujin
Program in Clinical Medicine, School of Medicine, Zhejiang University, Hangzhou, China.
Department of Hematology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China.
Ther Adv Hematol. 2022 Jan 31;13:20406207211072839. doi: 10.1177/20406207211072839. eCollection 2022.
MYD88/CD79B-mutated (MCD) genotype is a genetic subgroup of diffuse large B-cell lymphoma (DLBCL) with the co-occurrence of MYD88 and CD79B mutations. MCD genotype is characterized by poor prognosis and extranodal involvement especially in immune-privileged sites. MCD model is dominated by activated B-cell (ABC)-like subtype of DLBCLs. It is generally accepted that the pathogenesis of MCD DLBCL mainly includes chronic active B-cell receptor (BCR) signaling and oncogenic MYD88 mutations, which drives pathological nuclear factor kappa B (NF-κB) activation in MCD lymphoid malignancies. CD79B and MYD88 mutations are frequently and contemporaneously founded in B-cell malignancies. The collaboration of the two mutations may explain the unique biology of MCD. Meanwhile, standard immunochemotherapy combine with different targeted therapies worth further study to improve the prognosis of MCD, according to genetic, phenotypic, and clinical features of MCD type. In this review, we systematically described mechanism, clinical characteristics, and targeted therapy of MCD DLBCL.
MYD88/CD79B 突变型（MCD）基因型是弥漫性大 B 细胞淋巴瘤（DLBCL）的一个遗传亚组，同时存在 MYD88 和 CD79B 突变。MCD 基因型的特征是预后不良和结外受累，尤其是在免疫赦免部位。MCD 模型以 DLBCL 的活化 B 细胞（ABC）样亚型为主。一般认为，MCD DLBCL 的发病机制主要包括慢性活化 B 细胞受体（BCR）信号传导和致癌性 MYD88 突变，这在 MCD 淋巴恶性肿瘤中驱动病理性核因子κB（NF-κB）活化。CD79B 和 MYD88 突变在 B 细胞恶性肿瘤中经常同时出现。这两种突变的协同作用可能解释了 MCD 的独特生物学特性。同时，根据 MCD 型的遗传、表型和临床特征，标准免疫化疗联合不同的靶向治疗值得进一步研究以改善 MCD 的预后。在本综述中，我们系统地描述了 MCD DLBCL 的机制、临床特征和靶向治疗。
