title: Optimizing therapy for nodal marginal zone lymphoma.
abstract: Nodal marginal zone lymphoma (NMZL) is a rare form of indolent small B-cell lymphoma which has only been clearly identified in the last 2 decades and which to date remains incurable. Progress in therapeutic management has been slow, largely due to the very small number of patients treated and the heterogeneity of treatments administered; thus, standard-of-care treatment is currently nonspecific for this lymphoma entity. In this review, treatments routinely used to manage adult NMZL patients are presented, principally based on immunochemotherapy (when treatment is needed). Biological research behind the key axes of agents currently under development is described; development of novel agents is heavily based on data from gene profiling and genome-wide sequencing research, uncovering a number of critical deregulated pathways specific to NMZL tumors. These include B-cell receptor, JAK/STAT, NF-κB, NOTCH, and Toll-like receptor signaling pathways, as well as intracellular processes such as the cell cycle, chromatin remodeling, and transcriptional regulation in terms of epigenetic modifiers, histones, or transcriptional co-repressors, along with immune escape via T-cell-mediated tumor surveillance. These pathways are examined in detail and a projection of how the field may evolve in the near future for an efficient personalized treatment approach for NMZL patients is presented.
authors.affiliation: Hémato-Oncologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Sorbonne Paris Cité, Université Paris Diderot, Paris, France; Sorbonne Paris Cité, EA7324, Paris Descartes Université, Paris, France;
authors.forename: Catherine
authors.initials: C
authors.lastname: Thieblemont
authors.affiliation: Sorbonne Paris Cité, EA7324, Paris Descartes Université, Paris, France; Anatomo-Pathologie, Hôpital Necker, AP-HP, Paris, France;
authors.forename: Thierry
authors.initials: T
authors.lastname: Molina
authors.affiliation: Hématologie, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France; and UMR_S 1138, Université Pierre et Marie Curie, Paris, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Davi
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D000998:Antiviral Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D019468:Disease Management; D018450:Disease Progression; D005803:Genes, Immunoglobulin; D056726:Genetic Association Studies; D019698:Hepatitis C, Chronic / Q000150:complications / Q000188:drug therapy; D006801:Humans; D007167:Immunotherapy; D008198:Lymph Nodes / Q000473:pathology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D058990:Molecular Targeted Therapy; D009363:Neoplasm Proteins / Q000037:antagonists & inhibitors / Q000235:genetics; D011379:Prognosis; D011878:Radiotherapy; D015398:Signal Transduction / Q000187:drug effects; D059467:Transcriptome; D016896:Treatment Outcome
chemical_list: D000998:Antiviral Agents; D009363:Neoplasm Proteins