Splenic marginal zone lymphoma (SMZL) is an extremely rare indolent non-Hodgkin lymphoma (NHL) comprising about 0.9% of all NHL, with a median age of presentation being 69 years. We present a case of splenic marginal zone lymphoma in an elderly male presented with easy fatiguability and massive splenomegaly of one year duration. His haemogram and peripheral blood smear showed anemia with absolute lymphocytosis, presence of villous lymphocytes and thrombocytopenia. Bone marrow biopsy revealed increased lymphocytes and a diffuse pattern of marrow involvement. CD19, CD20 were positive on IHC while CD5, CD23, CD10, CD103 and cyclin D were negative. Bone marrow aspirate flow cytometry showed a CD5, CD10, CD103 negative kappa restricted B Cell lymphoproliferative disorder and based on characteristic morphology the diagnosis of a SMZL was made and started on Rituximab and Bendamustine.
SMZL is a low grade B cell neoplasm composed of small lymphocytes that originate from the splenic white pulp germinal centers. Patients usually present with splenomegaly and usually there is no lymphadenopathy. SMZL pathogenesis involves antigen or super antigen stimulation and molecular deregulation of different genes including NOTCH2 and KLF2. Many studies believe that its pathogenesis may be closely related to 7q distortion. The prognosis of SMZL is overall favorable with a 5-year survival of approximately 70% and median survival > 10 years. Mainstay of treatment is chemotherapy and if indicated, splenectomy.
SMZL presents many unsolved questions, such as standard prognostic criteria and standard treatment because the clinical, immunophenotypic and genetic features of SMZL are different from other marginal zone lymphomas and hence case reports that clarify many issues are important in the clinical practice

Keywords: Splenic Marginal Zone Lymphoma; Splenomegaly; Villous Lymphocytes; NOTCH2 Mutation; Indolent NHL

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Citation

Citation: Gurpreet Kaur., et al. ““Unveiling the Uncommon” Splenic Marginal Zone Lymphoma - A Rare Case Report with Review of Literature”.Acta Scientific Cancer Biology 8.1 (2024): 44-50.

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Copyright: © 2024 Gurpreet Kaur., et al. “Study of p16INK4a Immunostaining as Specific Biomarker in the diagnosis of Cervical Intraepithelial Neoplasia and This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.