Marginal zone B-cell lymphoma: lessons from Western and Eastern diagnostic approaches

Marginal zone B-cell lymphomas (MZLs) are a group of clinically indolent B-cell lymphomas postulated to derive from memory B lymphocytes in the 'marginal zone' of secondary lymphoid tissue. Today, MZL is recognised as a nosological umbrella term encompassing distinct entities with some shared phenotypic and genotypic features, including extranodal marginal zone B-cell lymphoma (EMZL) or mucosa-associated lymphoid tissue (MALT) lymphoma, splenic MZL, and nodal MZL, accounting for approximately 70%, 20%, and 10% of MZLs, respectively. These lymphomas share some phenotypic and genotypic features and have some variants and related provisional diseases, but are different in regards to their clinical and molecular characteristics. In addition, they are frequently associated with chronic antigenic stimulation represented either by infectious agents, particularly bacteria and viruses, or autoimmune diseases as exemplified by Sjögren syndrome, Hashimoto thyroiditis, and newly recognised IgG4-related disease. Furthermore, several chromosomal translocations have been identified in EMZL. In this review, we will focus on the updated histopathological criteria and the main problems with differential diagnoses in order to aid the diagnostic approach in our routine practice.