Marginal zone lymphoma (MZL) is the second most common form of indolent non-Hodgkin lymphoma. Protheragen is doing something about the challenges of managing MZL by focusing on advanced technologies and competent professionals that build innovative therapeutic options. With our all-encompassing support services, we will provide a clear uncomplicated pathway for you, from drug candidate development to market launch.
Marginal zone lymphoma (MZL) is a group of indolent B-cell non-Hodgkin lymphomas (NHLs) stemming from memory B cells situated in the marginal zone of lymphoid tissues. MZL constitutes 5-17% of worldwide NHL cases and is further subtyped into three types based on anatomical involvement:
| Subtypes | Primary Site | Genetic Alterations |
|---|---|---|
| Extranodal MZL (EMZL) | Extranodal sites (stomach, lung, ocular adnexa, etc.) | t(11;18)(API2-MALT1), trisomy 3/18, TNFAIP3 mutations |
| Splenic MZL (SMZL) | Spleen, bone marrow, peripheral blood | del(7q), NOTCH2, KLF2 mutations |
| Nodal MZL (NMZL) | Lymph nodes (no extranodal involvement) | Similar to SMZL/EMZL |
Marginal zone lymphoma (MZL) arises from antigen-stimulated memory B-cells in lymphoid marginal zones, driven by chronic inflammation, infections, or autoimmune triggers. Genetic aberrations, such as API2-MALT1 fusions in EMZL, NOTCH2 mutations in SMZL, and NF-κB pathway dysregulation across subtypes, promote B-cell survival and proliferation. The tumor microenvironment, enriched with T-cells and cytokines, further supports lymphomagenesis.
Fig.1 Pathogenesis of extranodal marginal zone lymphoma (EMZL). (Schreuder, Max I., et al., 2017)
| Therapy | Mechanism of Action | Targets | NCT Number | Research Phase |
|---|---|---|---|---|
| Ibrutinib + Rituximab | Blocks BCR signaling and depletes B cells. | BTK/CD20 | NCT03697512 | Phase II |
| Orelabrutinib + Obinutuzumab | Inhibits BTK and enhances antibody-dependent cellular cytotoxicity (ADCC). | BTK/CD20 | NCT06513234 | Phase II |
| Orelabrutinib + Obinutuzumab + Lenalidomide | Blocks B-cell receptor signaling, promotes B-cell exhaustion, and modulates immune responses. | BTK/CD20/Cereblon | NCT06454968 | Phase II |
| Obinutuzumab | Induces direct B-cell apoptosis and enhances ADCC. | CD20 | NCT03322865 | Phase II |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.
Recognizing the complexity of diagnosing and treating marginal zone lymphoma (MZL), Protheragen is committed to building a team of experts to provide cutting-edge diagnostic and therapeutic development solutions. Our commitment lies in providing a variety of customized therapy development services to meet the diverse research needs of our customers. We also excel in generating precise disease models that are carefully engineered to replicate the unique features of MZL. These models are valuable tools for validating the safety and efficacy of potential therapeutics.
At Protheragen, we are dedicated to supporting the development of innovative therapies through comprehensive preclinical research services. Our expertise spans pharmacodynamics (PD), pharmacokinetic (PK) and toxicology studies, ensuring a thorough evaluation of your therapeutic candidates. If you are interested in our services, please feel free to contact us for more details and quotation information of related services.
Reference
Myeloproliferative Disorders (MPDs)
Bone Marrow Failure Syndromes (BMFS)
