Article ; Online: Management of marginal zone lymphomas.
Hematology. American Society of Hematology. Education Program
2022 Volume 2022, Issue 1, Page(s) 676–687
Abstract: 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 initial assessment requires specific diagnostic and staging procedures depending ... ...
| Abstract | 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 initial assessment requires specific diagnostic and staging procedures depending on organ-related peculiarities. In particular, although positron emission tomography/computed tomography was not initially recommended, recent data have reassessed its role in the routine staging of MZL, especially when only localized treatment is planned or there is a suspicion of histologic transformation. Recent findings have improved the risk stratification of MZL patients, highlighting the association of early progression after frontline therapy with worse overall survival. A significant fraction of MZL cases may be related to specific bacterial (ie, Helicobacter pylori in gastric EMZL) or viral infections (hepatis C virus), and in the earlier phases of disease, a variable percentage of patients may respond to anti-infective therapy. Involved-site radiotherapy has a central role in the management of localized EMZL not amenable to or not responding to anti-infective therapy. Although rituximab-based treatments (bendamustine- rituximab in advanced EMZL or rituximab monotherapy in SMZL) have demonstrated favorable results, the current therapeutic scenario is predicted to rapidly change as emerging novel agents, especially Bruton's tyrosine kinase inhibitors, have demonstrated promising efficacy and safety profiles, leading to their approval in the relapsed setting. Moreover, a large variety of novel agents (phosphatidylinositol 3-kinase inhibitors, chimeric antigen receptor T-cells, bispecific antibodies) are being tested in MZL patients with encouraging preliminary results. |
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| MeSH term(s) | Humans ; Phosphatidylinositol 3-Kinases/therapeutic use ; Phosphoinositide-3 Kinase Inhibitors/therapeutic use ; Lymphoma, B-Cell, Marginal Zone/therapy ; Lymphoma, B-Cell, Marginal Zone/drug therapy ; Bendamustine Hydrochloride/therapeutic use ; Rituximab/therapeutic use |
| Chemical Substances | Phosphatidylinositol 3-Kinases (EC 2.7.1.-) ; Phosphoinositide-3 Kinase Inhibitors ; Bendamustine Hydrochloride (981Y8SX18M) ; Rituximab (4F4X42SYQ6) |
| Language | English |
| Publishing date | 2022-12-09 |
| Publishing country | United States |
| Document type | Journal Article ; Research Support, Non-U.S. Gov't |
| ZDB-ID | 2084287-9 |
| ISSN | 1520-4383 ; 1520-4391 |
| ISSN (online) | 1520-4383 |
| ISSN | 1520-4391 |
| DOI | 10.1182/hematology.2022000362 |
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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