Onkológia 5/2017

Marginal zone lymphoma

Marginal zone lymphomas (MZL) represent group of indolent lymphomas originating from post-germinal cells of the marginal zone of germinal follicles, in whose pathogenesis chronic antigenic stimulation plays an important role. Activation of the NF kappa-B pathway is characteristic feature in these lymphomas on the molecular level. An antibiotic (ATB) eradication of Helicobacter pylori infection is indicated in the treatment of extranodal lymphomas of the marginal zone of the gastric mucosal tissue with localized involvement. “Involved field” radiotherapy might be used in the treatment of limited stages of nongastric MALT lymphomas and nodal marginal zone lymphomas. Optimal treatment regimen in advanced stage disease remains to be defined, currently patients may be treated with rituximab monotherapy or immunochemotherapy with regard to disease burden, performance status and co-morbidities. In splenic marginal zone lymphoma, rituximab monotherapy is preferred first-line treatment. Immunomodulatory drug lenalidomide in combination with rituximab and agents affecting the B-cell receptor pathway (phosphatidyl-inositol-3 kinase and Bruton tyrosine kinase inhibitors) appear to be promising. In nongastric MALT lymphomas, ATB therapy targeting other possible etiological agents and treatment with high-dose clarithromycin are subject of research.

Keywords: marginal zone lymphoma, treatment, rituximab, imunochemotherapy