From the Guidelines
Marginal zone lymphoma (MZL) typically follows an indolent course with slow progression, and the treatment approach should prioritize a "watch and wait" strategy for asymptomatic patients, with intervention reserved for symptomatic disease or disease progression, as recommended by the ESMO clinical practice guidelines 1. The median survival for MZL patients ranges from 8-12 years, with many patients living much longer, and the disease remains manageable for extended periods with appropriate treatment and monitoring 1. When treatment is required, options include:
- Helicobacter pylori eradication therapy for gastric MZL, which can induce lymphoma regression and long-term clinical disease control in three-quarters of patients 1
- Radiotherapy (ISRT) for localized extragastric MZL (EMZL) 1
- Anti-HCV therapy for patients with HCV-associated lymphoma 1
- Chemotherapy, immunotherapy, or combination chemoimmunotherapy for patients with symptomatic disseminated disease, contraindications to RT, failure after antibiotics or after local therapy, or clinical suspicion of histological transformation, with options including R-chlorambucil, R-bendamustine, rituximab monotherapy, R-lenalidomide, and R-CHOP 1 Regular monitoring with clinical examinations, blood tests, and occasional imaging is essential throughout the disease course, with a recommended follow-up schedule of every 6 months for asymptomatic patients with disseminated MZL, and every 3 months for the first 2 years, and every 6 months thereafter for patients with EMZL at non-gastric sites 1. In patients with primary Sjögren's syndrome (pSjS) and MZL, a personalized therapeutic approach driven by the haematologist/oncologist is recommended, with consideration of a watchful waiting approach for low-grade lymphomas, and chemotherapy or radiotherapy for more aggressive disease or disseminated MALT lymphoma 1.
From the Research
Course of Marginal Zone Lymphoma
The course of marginal zone lymphoma can vary depending on the subtype and treatment approach.
- For gastric MALT lymphoma, the majority of patients have a favorable long-term outcome with exclusive Helicobacter pylori eradication therapy, offering a real chance of cure 2.
- The treatment of gastric MALT lymphoma typically involves antibiotics to eradicate H. pylori, with a recommended regimen including a proton pump inhibitor, clarithromycin, and amoxicillin (or metronidazole) 3.
- In areas with high resistance to clarithromycin, quadruple regimens with or without bismuth are recommended 4.
- The prognosis of marginal zone lymphoma can be predicted using models such as the MALT-IPI and the MZL-IPI, which incorporate factors such as elevated serum LDH, anemia, and nodal or disseminated subtypes 5.
- Treatment approaches for marginal zone lymphoma may include chemo-immunotherapy, radiotherapy, and emerging treatments such as BTK inhibitors for relapsed or refractory disease 5.
- The clinical course of marginal zone lymphoma is often indolent, with a prolonged clinical course, and may involve chronic infection with H. pylori or other pathogens 6.