Marginal Zone Lymphoma
Marginal Zone Lymphoma
Updated: 08/19/2024
© Jun Wang, MD, PhD
General features
- Usually indolent
- May involve various sites
Mucosal associated lymphoid tissue (MALT)
lymphoma: Most common
Nodal marginal zone lymphoma
Splenic marginal zone lymphoma
Cutaneous marginal zone lymphoma
- Stomach most common extranodal site, associated with H. Pylori
Clinical presentations
- Nonspecific, including lymphadenopathy, mass, etc
- Bone marrow/splenic involvement
Key pathogenesis
- Likely originated from B memory cells
- Associated with autoimmune disorders: Sjogren syndrome, Hashimoto thyroiditis, etc
- If H. pylori associated, may regress after eradication of H. pylori, unless with t(11;18)
Key morphological features of
tumor cells
- Larger lymphocytes
- Monocytoid or plasmacytoid neoplastic B cells
- Epithelial infiltrate
Markers
- Positive: CD19, CD20, light chain restriction
- Negative: CD5, CD10, CD23
Genetic abnormalities
- Various
- Most common: t(11;18)(q21;q21) API2-MALT1
Negative prognosis indicator
- t(11;18)
- Nuclear expression of NF kappa B
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