Marginal zone lymphomas

The three main types of marginal zone lymphoma (MZL), recognized by the current lymphoma classifications are the extranodal MZL of mucosa-associated lymphoid tissue, the splenic MZL, and the nodal MZL. They share some karyotype lesions (trisomies of chromosomes 3 and 18, deletions at 6q23), and alterations of the nuclear factor kappa B (NFkB) pathway are also common in all of them. However, they differ in the presence of recurrent translocations, mutations affecting the Notch signaling pathway (NOTCH2 and less commonly NOTCH1), the transcription factors Kruppel-like factor 2 (KLF2) or the receptor-type protein tyrosine phosphatase delta (PTPRD). This review summarizes the most recent and significant advances in our understanding of the epidemiology, genetics, and biology of MZLs and outlines the current principles of the standard management of MZL at different anatomic sites.

Automated summary

The three main types of marginal zone lymphoma (MZL) are extranodal MZL of mucosa-associated lymphoid tissue, splenic MZL, and nodal MZL. They share certain karyotype lesions and alterations in the NFkB pathway. However, they differ in the presence of recurrent translocations, mutations affecting the Notch signaling pathway, the transcription factors KLF2 or PTPRD. This review summarizes recent advances in understanding the epidemiology, genetics, and biology of MZLs, as well as the standard management principles.