MYD88L265P and CD79B double mutations type (MCD type) of diffuse large B-cell lymphoma: mechanism, clinical characteristics, and targeted therapy

Rongrong Chen; De Zhou; Lulu Wang; Lixia Zhu; Xiujin Ye

doi:10.1177/20406207211072839

MYD88^L265P and CD79B double mutations type (MCD type) of diffuse large B-cell lymphoma: mechanism, clinical characteristics, and targeted therapy

Ther Adv Hematol. 2022 Jan 31:13:20406207211072839. doi: 10.1177/20406207211072839. eCollection 2022.

Authors

Rongrong Chen¹, De Zhou², Lulu Wang¹, Lixia Zhu², Xiujin Ye³

Affiliations

¹ Program in Clinical Medicine, School of Medicine, Zhejiang University, Hangzhou, China.
² Department of Hematology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China.
³ Department of Hematology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310003, Zhejiang, China.

Abstract

MYD88/CD79B-mutated (MCD) genotype is a genetic subgroup of diffuse large B-cell lymphoma (DLBCL) with the co-occurrence of MYD88^L265P 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^L265P 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.

Keywords: CD79B; MYD88; NF-κB; chronic active BCR signaling; diffuse large B-cell lymphoma.

Publication types

Review