Management of marginal zone lymphomas

Marginal zone lymphomas (MZLs) represent about 7% of B-cell non-Hodgkin lymphomas and include 3 different subtypes- namely, extranodal (EMZL), nodal, and splenic (SMZL). The ini tial assess ment requires spe cifi c diag nos tic and stag ing procedures depending on organ-related peculiarities. In particular, although positron emission tomography/computed tomog ra phy was not ini tially recommended, recent data have reassessed its role in the rou tine stag ing of MZL, espe-cially when only local ized treat ment is planned or there is a sus pi cion of his to logic trans for ma tion. Recent find ings have improved the risk strat i fi ca tion of MZL patients, high light ing the asso ci a tion of early pro gres sion after front line ther apy with worse over all sur vival. A sig nifi cant frac tion of MZL cases may be related to spe cifi c bac te rial (ie, Helicobacter pylori in gas tric EMZL) or viral infec tions (hepatis C virus), and in the ear lier phases of dis ease, a var i able per cent age of patients may respond to anti -infec tive ther apy. Involved -site radio ther apy has a cen tral role in the man age ment of local ized EMZL not ame na ble to or not responding to anti -infec tive ther apy. Although rituximab -based treat ments (bendamustine - rituximab in advanced EMZL or rituximab monotherapy in SMZL) have dem on strated favor able results, the cur rent ther-a peu tic sce nario is predicted to rap idly change as emerg ing novel agents, espe cially Bruton ' s tyro sine kinase inhib i tors, have dem on strated prom is ing effi cacy and safety pro fi les, lead ing to their approval in the relapsed set ting. Moreover, a large variety of novel agents (phosphatidylinositol 3-kinase inhibitors, chimeric antigen receptor T-cells, bispecific anti-bodies) are being tested in MZL patients with encour ag ing pre lim i nary results.