From the Guidelines
Nodal marginal zone B-cell lymphoma (NMZL) with BCL6 expression should be treated with rituximab-based chemotherapy regimens, such as R-CHOP or R-bendamustine, for advanced disease, while localized disease may be managed with radiation therapy. The diagnosis and treatment of NMZL are guided by specific criteria, including the achievement of complete remission (CR) defined by normal spleen size, normal blood counts, negative flow cytometry on blood, and negative immunohistochemistry (IHC) on bone marrow biopsy 1.
Key Considerations
- NMZL is a rare type of non-Hodgkin lymphoma that primarily affects lymph nodes
- BCL6 expression is noteworthy as it's typically associated with more aggressive lymphomas and may influence prognosis
- The standard R-CHOP regimen is administered every 21 days for 6 cycles, with rituximab 375 mg/m², cyclophosphamide 750 mg/m², doxorubicin 50 mg/m², vincristine 1.4 mg/m² (capped at 2 mg), and prednisone 100 mg daily for 5 days
- Regular follow-up with CT or PET scans every 3-6 months for the first 2 years after treatment is recommended, then annually for at least 5 years
Treatment Response and Prognosis
- Treatment response is generally favorable with 5-year overall survival rates of approximately 70-80%
- NMZL has an indolent course but can transform to more aggressive types, highlighting the importance of regular follow-up and monitoring 1
Relevant Guidelines
- The ESMO clinical practice guidelines for diagnosis, treatment, and follow-up of marginal zone lymphomas provide specific recommendations for SMZL and NMZL, including response assessment criteria and treatment options 1
From the Research
Nodal Marginal Zone Lymphoma and BCL6
- Nodal marginal zone lymphoma (NMZL) is a rare type of lymphoma, accounting for about 10% of marginal zone lymphoma cases 2
- The clinical features and treatment outcomes of NMZL have not been well defined due to its rarity 3
- BCL6 is a protein that plays a crucial role in the development and progression of various types of lymphoma, including NMZL 4
- Overexpression of BCL6 has been associated with aggressive behavior and poor prognosis in some types of lymphoma 4
Treatment Outcomes and Prognosis
- The treatment outcomes and prognosis of NMZL vary depending on the stage and other factors, such as performance status and follicular lymphoma international prognostic index (FLIPI) 3
- Rituximab maintenance therapy has been shown to improve progression-free survival (PFS) in patients with stage III-IV marginal zone lymphoma, including those with NMZL 5
- The choice of front-line chemotherapy may impact survival in older patients with indolent lymphomas, including NMZL 6
BCL6 Expression in NMZL
- BCL6 expression has been reported in some cases of NMZL, particularly those with aggressive behavior and complex chromosomal changes 4
- The overexpression of BCL6, along with other proteins such as MYC and BCL2, may contribute to the aggressive behavior and poor prognosis of some NMZL cases 4
- Further studies are needed to fully understand the role of BCL6 in NMZL and its potential as a therapeutic target 4