Overview on the management of non-gastric MALT lymphomas.

ABSTRACT

Extranodal marginal zone B-cell lymphomas (EMZLs) of the mucosa-associated lymphoid tissue (MALT) are indolent lymphomas which can present at any extranodal site. The most frequent localizations (other than stomach) are ocular adnexa, salivary gland, skin, lung and thyroid. Chronic inflammation and antigenic stimulation are a potential risk for the development of MALT lymphomas. While Helicobacter Pylori (HP) is known to be associated with gastric MALT lymphoma and antibiotic therapy is effective in the setting of HP-positive, other microorganisms (such as Chlamydophila Psittaci, Campylobacter Jejiuni, Borrelia Burgdoferi) have been implicated in the pathogenesis of non-gastric MALT lymphomas. However, antibiotic therapy has not been extensively investigated for the non-gastric type, except for ocular adnexal MALT lymphoma, which could benefit from an upfront treatment with doxycycline. Surgery, radiotherapy, Rituximab alone or in combination with chemotherapy and "chemo-free" approaches, including lenalidomide, have shown efficacy in the treatment of non-gastric MALT lymphomas.