Nodal marginal zone lymphoma

Abstract

Nodal marginal zone lymphoma (NMZL) is a primary nodal B-cell tumor originating in the marginal zone of the lymph node and without clinical evidence of extranodal or splenic disease. This lymphoma was initially named monocytoid B-cell lymphoma (MBCL) due to monocytoid features of the tumor cells. According to the World Health Organization (WHO) classification, NMZL is a distinctive disease entity but with histologic and genetic similarities with the other two clinicopathological subtypes of MZL, extranodal MZL of mucosa-associated lymphoid tissue (MALT) type and splenic MZL, but with different and more aggressive clinical ?ndings and a shorter survival time. NMZL is clinically similar to other low-grade (indolent) nodal lymphomas, such as follicular or small lymphocytic lymphomas. A clinical, morphological, and immunoarchitectural spectrum is seen also within the NMZL entity; why a careful evaluation of the disease is important before clinical decisions. The choice of optimal therapy for patients with NMZL represents a dilemma, since only small retrospective series and few clinical studies have been published.