“Lien Vasti”- A Case of Splenic Marginal Zone Lymphoma

Splenic marginal zone lymphoma (SMZL) is a rare and uncommon subtype of B cell Non Hodgkin’s lymphoma (NHL) that may present with marked splenomegaly with CD20 rich B-cells in blood and bone marrow and absent generalized lymphadenopathy. Here, we present a case of massive splenomegaly with pancytopenia, normal peripheral blood smear (PBS) and bone marrow picture, with generalized lymphadenopathy.

Case Capsule: A 63-year-old lady, presented with hepatosplenomegaly, loss of appetite & weight for 4 years, with generalized lymphadenopathy for 2 months. PBS and bone marrow aspiration revealed simple pancytopenia and normal picture. Intra operatively, the spleen measured 43 centimetres (cms) in (length) and weighed 7.2 kilograms (kgs), making it the longest as well as the largest spleen ever surgically removed, in Asia and second largest in the world. The immuno-histochemistry (IHC) was quite consistent, with splenic marginal zone lymphoma.

Conclusions: SMZL is a rare condition, with an unexpected course that leads to progressive splenomegaly. In a case of SMZL, without blood and bone marrow involvement with symptoms secondary to massive splenomegaly and hypersplenism, upfront splenectomy rapidly alleviates symptoms of hypersplenism and further, aids in histological diagnosis for systemic chemotherapy. Management of SMZL requires a multidisciplinary approach with chemotherapy +/- splenectomy to achieve optimal outcomes.