Primary Splenic Diffuse Large B-Cell Lymphoma with Massive Splenomegaly

Primary malignancies of the spleen are very rare, diffuse large B-cell lymphoma of the spleen is a rare primary neoplasm. Primary splenic diffuse large B-cell lymphoma is a type of non-Hodgkin’s lymphoma that constitute, less than 1% of non -Hodgkin’s lymphoma and less than 2% of all lymphomas. Primary splenic lymphoma has been associated with the hepatitis C and HIV virus. It affects men more than women and present in late middle age.

Patient with diffuse large B cell lymphoma typically present with fever, fatigue, weight loss, palpable splenomegaly and left upper quadrant pain. Diffuse large B cell lymphoma stage I being limited to the spleen, whereas stage II spleen and hilar Lymph-node involvement and stage III is distant metastasis including liver and surrounding organs involvements, stomach, pancreas, kidney and colon. Stage I disease, a splenectomy is the standard treatment for DLBSL. Radiation and chemotherapy can be used. When the cancer is confined to the spleen, the prognosis is quite favourable with a median survival of 7.48 years.

We are reporting an extremely rare case of primary splenic lymphoma in a 45 years old female patient, presented with abdominal pain and huge splenomegaly. The CT revealed a bulky, low density, irregular mass in the spleen and clinically disease limited to spleen only. So we performed open total splenectomy.