A 78year old female presented with complaints of easy fatiguability, abdominal distension, breathlessness on exertion, loss of appetite and weight for the past two months. On examination, she was found to have massive splenomegaly. PET-CT showed massive splenomegaly with diffuse splenic and marrow uptake. Bone marrow aspiration and biopsy findings were suggestive of chronic lymphoproliferative disorder. Flow cytometry was positive ,suggestive of non CLL B-cell chronic Lymphoproliferative disorder. HIV, HbsAg and HCV were negative. Patient was diagnosed to have splenic marginal zone lymphoma and risk stratified as severe with low Hb, low platelets and elevated serum LDH levels. Patient was started on Rituximab weekly for 6 weeks as induction therapy. This case is being presented for its ,rarity and unique presentation.

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