Marginal zone lymphomas

Abstract

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.

Characterization of Human B Cell Hematological Malignancies Using Protein-Based Approaches

“…Symptomatic, advanced-stage MZL is best managed with rituximab-based therapies. In cases of severely symptomatic or bulky disease, combination immunochemotherapy with chlorambucil or bendamustine may be preferred over rituximab alone [60].…”

A Phase II Study of 131I-rituximab for Patients With Relapsed or Refractory Marginal Zone Lymphoma

“…1 , 2 The 3 most typical manifestations of MZL are extranodal marginal zone lymphoma (EMZL), nodal marginal zone lymphoma (NMZL), and splenic marginal zone lymphoma (SMZL). 3 , 4 Owing to its indolent course of presentation, most patients with MZL have a favorable prognosis compared with those with aggressive lymphomas. The 5-year overall survival (OS) rate is approximately 80% after diagnosis.…”