How I treat MALT lymphoma: ‘a subjective interpretation of the gospel according to Isaacson….’

Abstract

Mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) is an indolent B-cell lymphoma characterised by a fascinating interplay between chronic antigenic stimulation, an immune response insufficient for elimination of the antigen and a mucosal ‘battleground’. The archetype of this association is infection of the gastric mucosa with Helicobacter pylori (HP): a single course of antibiotic HP-eradication treatment may result in long-term remission in up to 80% of patients and is the gold standard for first-line therapy of HP-associated gastric MALT lymphoma. In extragastric or disseminated disease, treatment options range from wait and see in asymptomatic individuals to radiotherapy in localised stages, anti-CD20-antibodies in patients with low symptomatic burden and chemotherapy-based treatment or radio-immunotherapy in symptomatic disease. In addition, more refined immunomodulatory strategies beyond simple eradication of bacteria such as long-term use of the macrolide clarithromycin or the immunomodulatory drug lenalidomide are active. In view of the indolent clinical course, the least toxic individual treatment should be chosen in a disease usually not influencing overall survival in affected patients.

Primary Hepatic Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue in a Patient with Chronic Hepatitis B Virus Infection: Case Report and Summary of the Literature

“…Two cases were closely observed under conservative treatment. As the eradication of HP results in long-term remission in up to 80% of patients for gastric MALT lymphoma, it should be considered the first treatment step regardless of disease stage [ 2 , 40 ]. The clinical efficacy of direct-acting antivirals has also been documented in patients with HCV-associated non-Hodgkin lymphoma for viral eradication therapy [ 20 , 22 ].…”

Two rare cases of bronchus‐associated lymphoid tissue lymphoma successfully treated with rituximab‐bendamustine

“…Indeed, many therapeutic options are available including surgery, radiotherapy, immunotherapy, and chemotherapy. 10 , 30 , 31 In many MALT lymphoma patients, there is a history of a chronic inflammatory disorder resulting in the accumulation of extranodal lymphoid tissue. The chronic inflammation may be the result of an infection, autoimmunity, or other unknown stimuli.…”

Gastric mucosa-associated lymphoid tissue lymphoma with central nervous system involvement: a case report and 8-year follow-up

“…MALT lymphoma is an indolent B-cell lymphoma characterized by a fascinating interplay between chronic antigenic stimulation, and immune response insufficient for the elimination of the antigen and a mucosal ‘battleground’. Therefore, it may have the possibility of relapse ( 18 ). In case of relapse, various therapies such as targeted therapies (novel agents targeting BTK and Pi3K) or the use of immunomodulatory agents may be considered to tackle the situation.…”

“…In case of relapse, various therapies such as targeted therapies (novel agents targeting BTK and Pi3K) or the use of immunomodulatory agents may be considered to tackle the situation. For instance, due to the role of the NF-kB pathway in the pathogenesis of MALT lymphoma, the proteasome inhibitor bortezomib can be considered to treat the MALT ( 18 ). Given the dependency of MALT lymphoma cells on the tumor microenvironment, this specific entity appears highly suitable for immunomodulatory treatment strategies ( 19 ).…”