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A Single-center Experience in Splenic Diffuse Red Pulp Lymphoma Diagnosis
Hunan L Julhakyan1, L S Al-Radi1, T N Moiseeva1
1National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
Clinical Lymphoma, Myeloma & Leukemia
|May 2, 2016
Summary
Splenic diffuse red pulp lymphoma (SDRPL) is a rare B-cell lymphoma presenting with significant splenomegaly and lymphocytosis. Diagnosis requires careful immunophenotyping, with splenectomy being the standard treatment.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- The 2008 WHO classification introduced splenic diffuse red pulp lymphoma (SDRPL), a rare entity with overlapping features with splenic marginal zone lymphoma and hairy cell leukemia (HCL).
- Limited clinical experience exists for SDRPL diagnosis and differential diagnosis among hematologists.
- This report aims to characterize the clinical and immunomorphologic features of SDRPL based on original observations.
Purpose of the Study:
- To describe the clinical and immunomorphologic characteristics of splenic diffuse red pulp lymphoma (SDRPL).
- To aid in the differential diagnosis of SDRPL from similar B-cell lymphomas.
- To report on the outcomes of patients diagnosed with SDRPL.
Main Methods:
- Analysis of 87 spleen specimens from B-cell lymphomas between 2013-2014.
- Diagnosis of SDRPL based on morphologic, immunohistochemical, immunophenotypic, and molecular examination of spleen, blood, and bone marrow samples.
- Characterization of circulating lymphocyte phenotype and bone marrow infiltration.
Main Results:
- SDRPL was diagnosed in 4.6% of cases, characterized by significant splenomegaly (average spleen weight 3900g) and high lymphocytosis (56-94%).
- Circulating lymphocytes exhibited a specific phenotype (CD20+ bright, CD11c+/±, CD103+/±, LAIR-1+, CD25-, CD5-, CD10-, CD23-). BRAFV600E mutation was negative.
- Bone marrow showed minor infiltration of lymphoid cells with a distinct immunophenotype. Patients achieved remission after splenectomy +/- chemotherapy.
Conclusions:
- Splenic diffuse red pulp lymphoma is a rare lymphoma suspected in cases with marked splenomegaly and lymphocytosis featuring villous lymphocytes with partial HCL markers and minimal bone marrow involvement.
- Splenectomy is the standard diagnostic and therapeutic approach for SDRPL.
- While differential diagnosis from SMZL and HCL is established, criteria for differentiating SDRPL from variant HCL require further investigation.
