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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
PubMed
Summary

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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.

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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.