From the Guidelines
The rate of CNS response after treatment for CNS marginal zone lymphoma is generally favorable, with complete response rates ranging from 58-69% depending on the treatment regimen used, as seen in recent studies such as the HOVON 105-ALLG NHL 24 trial and the PRECIS trial 1. CNS recovery typically does occur in most patients, though the timeline varies from weeks to months following successful treatment. First-line therapy usually consists of high-dose methotrexate combined with rituximab, often administered every 2-3 weeks for 4-6 cycles, with regimens such as MATRix, MBVP, or ReMBVP being commonly used 1. Some key points to consider in treatment include:
- The use of rituximab in induction combinations remains a matter of debate, with its efficacy and tolerability being discussed with patients and their carers 1.
- Reduced-dose whole-brain radiotherapy (WBRT) may be considered after chemotherapy completion for patients with CNS MZL 1.
- The recovery process involves gradual improvement in neurological symptoms, with cognitive functions often taking longer to fully recover than motor functions.
- Regular neurological assessments and MRI monitoring are essential during and after treatment to evaluate response and detect any early signs of recurrence.
- Treatment-related neurotoxicity can occur and may temporarily mask improvement, particularly in elderly patients or those receiving combined modality therapy. The indolent nature of marginal zone lymphoma contributes to its generally good response to treatment compared to more aggressive CNS lymphoma subtypes. In terms of specific treatment regimens, the MATRix regimen has been associated with efficacy and tolerability comparable to those reported in the IELSG32 trial, with a 2-year OS rate of 64% and a 6% treatment-related mortality (TRM) rate 1. Other regimens, such as MBVP and ReMBVP, have also shown promising results, with a 1-year PFS rate of 58% and a 3-year OS rate of 61% in the HOVON 105-ALLG NHL 24 trial 1. Overall, the choice of treatment regimen should be individualized based on patient factors, such as age, performance status, and comorbidities, as well as the specific characteristics of the lymphoma.
From the Research
Central Nervous System Response and Recovery
- The rate of central nervous system (CNS) response after treatment for CNS marginal zone lymphoma is relatively favorable, with an estimated 2-year progression-free survival (PFS) rate of 59% and overall survival (OS) rate of 80% 2.
- A study of primary CNS marginal zone lymphoma found that patients can exhibit an excellent prognosis after varied treatments, such as surgery, radiotherapy, chemotherapy, or their combinations 3.
- Another study reported a 3-year PFS rate of 81% and a 3-year OS rate of 90% in patients with stage III-IV marginal zone lymphoma who received rituximab-maintenance therapy following first-line R-CVP treatment 4.
Treatment Outcomes and Prognosis
- Extranodal marginal zone lymphoma of the central nervous system (CNS EMZBL) is a rare disease with a good treatment outcome and prognosis, with 77% of patients experiencing complete remission 5.
- A study of treatment of initial parenchymal CNS involvement in systemic aggressive B-cell lymphoma found that patients had a 3-year PFS rate of 45% and a 3-year OS rate of 49% 6.
- The prognosis of CNS marginal zone lymphoma appears to be favorable, particularly among patients with dural presentation and primary CNS presentation 2.
Factors Affecting Treatment Outcomes
- Elevated LDH and the presence of B symptoms were found to be significant prognostic factors for PFS in patients with stage III-IV marginal zone lymphoma 4.
- The site and extent of disease, its resectability, and the expected adverse effects of systemic therapy should be considered when developing individualized management plans for patients with CNS EMZBL 5.