Key points are not available for this paper at this time.
Abstract: Marginal zone lymphomas (MZLs) are a diverse group of indolent non-Hodgkin lymphoma (NHL) sharing a similar immunophenotype but exhibiting unique biology, clinical features and requiring specific treatment approaches. Three subtypes are recognized in the World Health Organization classification including extranodal MZL (EMZL), splenic MZL (SMZL) and nodal MZL (NMZL). MZL represents 10.5% to 11.8% of all B-cell lymphomas and most patients experience long survival. However, there is a subgroup of patients characterized by a more aggressive disease at risk for unfavorable outcomes. Over the last years an effort toward better understanding and identification of these patients was made. Several high-risk features and prognostic models were built to better identify patients at risk for shorter survival earlier in the disease course. However, compared to other low-grade lymphomas, like follicular lymphoma (FL), both survival risk prediction and treatment strategies to overcome poor outcomes have not been optimized yet. The incidence of higher grade transformation (HGT) in MZL is lower than in FL and may occur in all MZL subtypes, however, higher frequency of this event has been observed in SMZL and NMZL compared to EMZL. HGT is an independent risk factor associated with poor outcomes and current knowledge on biology, risks factors and treatment in patients experiencing transformation remains limited. Most recommendations are extrapolated from transformed FL with very few studies assessing treatment strategies in this population. In this review we aim to discuss the current state of the art in prognostic factors and risk stratification in MZL as well as HGT of these lymphomas. We also provide a rational approach to treat MZL patients undergoing HGT.
Alderuccio et al. (Tue,) studied this question.