Nodal and splenic marginal zone B cell lymphomas.

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Splenic marginal zone lymphoma (SMZL) and nodal marginal zone lymphoma (NMZL) are distinct, rare lymphoma subtypes.
• Accurate diagnosis is challenged by low reproducibility, necessitating integrated diagnostic approaches.
• Understanding the molecular and clinical features is crucial for improved diagnosis and treatment.

Purpose of the Study:

• To review current knowledge on SMZL and NMZL diagnosis and understanding.
• To highlight the role of molecular and clinical studies in differentiating these lymphomas.
• To discuss diagnostic challenges and the need for specific therapeutic and prognostic factors.

Main Methods:

• Review of morphological, immunophenotypical, and molecular characteristics of SMZL and NMZL.
• Analysis of clinical features and disease manifestations.
• Discussion of differential diagnoses, particularly with follicular lymphoma.

Main Results:

• SMZL involves the spleen, bone marrow, and peripheral blood; 7q allelic loss is a frequent molecular alteration.
• NMZL originates in lymph nodes, lacking extranodal or spleen involvement, with diagnostic challenges due to non-specific markers.
• Both are generally indolent, but aggressive courses can occur; molecular studies offer potential diagnostic and therapeutic targets.

Conclusions:

• Integrated diagnostic approaches combining morphology, immunophenotype, and molecular data improve SMZL and NMZL diagnosis.
• Further research into specific molecular markers and therapeutic strategies is essential for both SMZL and NMZL.
• Defining precise prognostic factors and therapeutic protocols is critical for optimal patient management.