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Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
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Secondary organs, including lymph nodes, the spleen, and mucosa-associated lymphoid tissue (MALT), work harmoniously to protect us from disease and infection.
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A Review on Splenic Diffuse Red Pulp Small B-Cell Lymphoma.

Elif Yilmaz1, Arashpreet Chhina2, Victor E Nava2

  • 1Department of Hematology and Oncology, Georgetown University Medical Center, Washington, DC 20007, USA.

Current Oncology (Toronto, Ont.)
|December 23, 2021
PubMed
Summary

Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare non-Hodgkin lymphoma. Diagnosis relies on splenic tissue or blood/marrow studies, with characteristic immunophenotype and low proliferation index aiding identification.

Keywords:
B-cell lymphomanon-Hodgkin lymphomaprimary splenic lymphomasplenic diffuse red pulp lymphomavillous lymphocytes

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Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare non-Hodgkin lymphoma (NHL), comprising less than 1% of cases.
  • Clinical presentation often includes splenomegaly, lymphocytosis, and hemocytopenia.
  • Diagnostic challenges arise due to overlapping features with splenic marginal zone lymphoma (SMZL) and hairy cell leukemia (HCL).

Purpose of the Study:

  • To delineate the diagnostic criteria and immunophenotypic characteristics of SDRPL.
  • To differentiate SDRPL from other small B-cell lymphomas.
  • To outline current treatment strategies for SDRPL.

Main Methods:

  • Analysis of splenic tissue, peripheral blood, and bone marrow studies.
  • Immunophenotypic profiling including CD markers, DBA.44, IgG, and cyclin D3 expression.
  • Assessment of Ki-67 proliferative index.

Main Results:

  • SDRPL shows diffuse red pulp involvement by monomorphic small B lymphocytes.
  • Characteristic immunophenotype: CD20+, DBA.44 (20-90%)+, IgG+, typically negative for CD5, CD10, CD23, cyclin D1, and others.
  • Low Ki-67 index and positive Cyclin D3 expression are key distinguishing features.

Conclusions:

  • Diagnosis of SDRPL requires careful integration of clinical, morphological, and immunophenotypic data.
  • Splenic tissue biopsy is the gold standard, but peripheral blood and bone marrow analysis can be sufficient.
  • Treatment options include splenectomy, rituximab, or chemoimmunotherapy for advanced disease.