Marginal zone lymphoma: old, new, targeted, and epigenetic therapies

Abstract

Marginal zone lymphoma (MZL) is an indolent B-cell lymphoma arising from marginal zone B-cells present in lymph nodes and extranodal tissues. MZL comprises 5-17% of all non-Hodgkin's lymphomas in adults. The World Health Organization categorizes MZL into three distinct types based on their site of impact: (1) splenic marginal zone lymphoma (SMZL); (2) nodal marginal zone lymphoma (NMZL); (3) extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, which can be subdivided into gastric and nongastric. The subgroups of MZL share some common features but are different in their biology and behavior. Owing to the rarity of MZL there are few randomized trials available comparing various treatment options and therefore treatment is controversial, lacking standard guidelines. Treatment should be patient tailored and can range from a 'watchful waiting' approach for asymptomatic patients without cytopenias to surgery or localized radiation therapy. Rituximab in combination with chemotherapy has resulted in longer failure-free survival than chemotherapy alone in patients with SMZL. Helicobacter pylori positive gastric MALT shows a good response rate to triple antibiotic therapy. Newer therapies such as bendamustine, everolimus, lenalidomide, vorinostat and phosphoinositide 3-kinase inhibitors are in clinical trials for patients with relapsed or refractory MZL and have shown promising results. We are presently conducting clinical trials testing the efficacy of the epigenetic activity of cladribine as a hypomethylating agent in combination with the histone deacetylase inhibitor (HDACi) vorinostat and rituximab in patients with MZL. Further studies with the newer agents should be done both in newly diagnosed or relapsed/refractory MZL to streamline the care and to avoid the use of toxic chemotherapies as initial treatment.

A case report of nodal marginal zone lymphoma: Diagnosis and management

“…The 5-year survival is 55%-79%. [2] This patient is a 58-year-old female with stage III disease and presented with prominent neck node mass which is most common site for involvement. Our patients' histopathological findings including morphological features and CD expression were similar to those of previously published literatures.…”

Splenectomy in Lymphoproliferative Disorders: A Single Eastern European Center Experience

“…Although splenic involvement can occur in any type of lymphoma, it is mostly seen in indolent/low-grade non-Hodgkin lymphomas (NHLs)/chronic lymphoproliferative disorders (CLPDs) like marginal zone and follicular lymphomas, hairy cell leukemia, and chronic lymphocytic leukemia/small lymphocytic lymphoma. Marginal zone lymphomas (MZLs) are a group of indolent B cell CLPDs, representing about 10% of all adult lymphomas; these are generated by the proliferation of malignant cells with a normal counterpart in B lymphocytes present in a particular peripheral area of the lymphoid follicle, the marginal zone [11]. MZL is further sub-classified as splenic marginal zone lymphoma (SMZL), usually presenting with massive isolated splenomegaly, nodal MZL, and extranodal MZL MALT type [12].…”

Pulmonary MALT lymphoma – when an opacity persists too long: a case report and literature review

“…There are many therapeutic options but no standard guidelines, owing to the rarity of this lymphoma. For localized disease, most frequently, radiotherapy is the first-line treatment 2,9 . Other options include surgical resection or "watchfull waiting" strategies for asymptomatic patients without cytopenias.…”

“…Tissue biopsy is the gold standard for diagnosis 1 . There is no clear consensus on treatment: localized disease can be treated with radiotherapy, surgery or observation while diffuse lymphomas are almost always managed with chemotherapy, either with a single or multiple agents 1,2 .…”