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[Experience in investigating splenic red pulp lymphoma].
L S Al-Radi1, T N Moiseeva1, H L Julhakyan1
1National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia.
Terapevticheskii Arkhiv
|April 13, 2016
Summary
Splenic red pulp lymphoma (SRPL) is a rare condition characterized by significant splenomegaly and lymphocytosis with specific hairy lymphocyte markers. Diagnosis and treatment often involve splenectomy, with clear criteria for differentiating it from hairy cell leukemia (HCL).
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Splenic red pulp lymphoma (SRPL) is a rare B-cell lymphoproliferative disorder.
- Accurate diagnosis and differentiation from similar conditions are crucial for effective management.
Purpose of the Study:
- To generalize hematologists' experience in diagnosing and differentially diagnosing SRPL.
- To establish diagnostic criteria for SRPL based on morphological, immunophenotypic, and molecular findings.
Main Methods:
- Examination of 87 splenic biopsy specimens from patients with B-cell lymphoproliferative diseases.
- Utilized morphological, immunohistochemical, immunophenotypic, and molecular analyses.
- Included blood and bone marrow examinations for diagnosis in select cases.
Main Results:
- SRPL cases presented with significant splenomegaly and lymphocytosis.
- Hairy lymphocytes exhibited specific phenotypes (CD20+, CD11c+/±, CD103+/±, LAIR-1+), lacked tartrate-resistant acid phosphatase, and BRAFV600E mutation.
- Bone marrow showed minimal lymphoid infiltration with distinct markers.
- Spleen tissue displayed red pulp infiltration with specific phenotypes (CD20+, DBA.44+, CD25-, Annexin1-, Cyclin D1-).
Conclusions:
- SRPL should be suspected in cases of significant splenomegaly and lymphocytosis with atypical hairy lymphocytes and minor bone marrow involvement.
- Splenectomy is the standard for diagnosis and treatment.
- Clear diagnostic criteria exist for differentiating SRPL from classical hairy cell leukemia (HCL).
