Splenic marginal zone lymphoma: a rare cause of splenomegaly

Splenic Marginal Zone Lymohoma(SMZL) is a very rare variety of lymphomas, accounting for only to 2% of NHL’s (1,3) which has an incidence of 0.015% in India(2) .It originates from the memory B lymphocyte in marginal zone of secondary lymphoid follicle. Present as an indolent or disseminated disease at the time of diagnosis, with mass per abdomen, early satiety, incidentally detected altered blood counts. With a limited treatment options available like splenectomy, rituximab with or without chemotherapy, SMZL poses great challenge in diagnosis also. 65 year old gentleman, diagnosed as SMZL with symptomatic splenomegaly, underwent a splenectomy. IHC reports suggesting CD5(-), which is present only in 15 to 25% of the cases(4) of SMZL. In a developing country with restrained economic conditions, splenectomy can be a good diagnostic as well as treatment modality, as there is no consensus regarding the best treatment available at present.