A Review on Splenic Diffuse Red Pulp Small B-Cell Lymphoma

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.