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Splenic marginal zone lymphoma with or without plasmacytic differentiation.
J P Duong Van Huyen1, T Molina, A Delmer
1Department of Pathology, H tel Dieu, AP-HP, Paris, France.
The American Journal of Surgical Pathology
|December 16, 2000
Summary
Splenic marginal zone lymphomas present with enlarged spleens. Some cases show lymphoplasmacytic components linked to autoimmune disorders and monoclonal proteins, distinct from Waldenstrom's macroglobulinemia.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Splenic marginal zone lymphomas (SMZL) are a heterogeneous group of B-cell lymphomas.
- Understanding the distinct clinicopathological features of SMZL is crucial for accurate diagnosis and prognosis.
Purpose of the Study:
- To characterize a series of 31 splenic marginal zone lymphoma cases.
- To investigate the clinical and pathological differences between SMZL with and without a lymphoplasmacytic component.
Main Methods:
- Retrospective analysis of 31 cases of splenic marginal zone lymphoma.
- Histopathological examination, including morphology and immunohistochemistry (CD20, CD45 RA, bcl-2, IgD).
- Correlation of clinicopathological findings with serological and autoimmune parameters.
Main Results:
- Two groups identified: Group A (with lymphoplasmacytic component) and Group B (without).
- Group A showed higher incidence of monoclonal serum component and autoimmune disorders (e.g., autoimmune hemolytic anemia).
- Seven cases met criteria for Waldenstrom's macroglobulinemia; lymphoma cells expressed CD20, CD45 RA, bcl-2, and variable IgD.
Conclusions:
- Splenic marginal zone lymphomas exhibit distinct subtypes based on lymphoplasmacytic component presence.
- The presence of a lymphoplasmacytic component is associated with specific clinical features, including autoimmune phenomena.
- SMZL generally shows slow progression, but transformation to large B-cell lymphoma can occur.