![]() 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. Helicobacter pylori positive gastric MALT shows a good response rate to triple antibiotic therapy. Rituximab in combination with chemotherapy has resulted in longer failure-free survival than chemotherapy alone in patients with SMZL. Treatment should be patient tailored and can range from a ‘watchful waiting’ approach for asymptomatic patients without cytopenias to surgery or localized radiation therapy. Owing to the rarity of MZL there are few randomized trials available comparing various treatment options and therefore treatment is controversial, lacking standard guidelines. The subgroups of MZL share some common features but are different in their biology and behavior. 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. MZL comprises 5–17% of all non-Hodgkin’s lymphomas in adults. Marginal zone lymphoma ( MZL) is an indolent B-cell lymphoma arising from marginal zone B-cells present in lymph nodes and extranodal tissues. Joshi, Monika Sheikh, Hassan Abbi, Kamal Long, Sarah Sharma, Kamal Tulchinsky, Mark Marginal zone lymphoma: old, new, targeted, and epigenetic therapies Much of the approach to managing transformed MZL is extrapolated from other indolent NHLs. Optimal treatment of HT of MZL remains unknown. However, recent advances in the understanding of molecular and genetic features of MALT have contributed to an evolving appreciation of HT in this disease. There are few data directly associated with risk and prognosis of transformation in MZL. Histologic transformation (HT) to an aggressive lymphoma is a rare event that can occur in any subtype, and at lower frequency compared to other indolent non Hodgkin lymphomas (NHL) like follicular lymphoma. ![]() ![]() ![]() Marginal zone lymphomas ( MZL) are a diverse group of indolent lymphoproliferative disorders that comprise three subtypes: nodal, splenic and mucosal associated marginal zone lymphomas (MALT). Transformation of marginal zone lymphoma (and association with other lymphomas). ![]()
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