Unusual Cause of Splenomagaly

Abstract

A 67-year-old man with a history of hypertension, presented with a 3-month history of fatigue, intermittent fever and abdominal pain over the left upper quadrant. Abdominal examination revealed a splenomegaly (4cm from costal arch), without any regional lymphadenopathy. The hemogram was normal and blood biochemistry did not show abnormalities. Abdominal sonography showed multiple hypoechoic splenic nodules. Abdominal CT revealed multiple hypodense splenic tumors, likely perisplenic lymphadenopathy, around the pancreatic tail and splenic hilum, involving the splenic vein (Figure 1). The patient underwent splenectomy, and perisplenic and peripancreatic lymphnode dissection (Figure 2). Pathology revealed malignant splenic marginal zone B-cell lymphoma (SMZL). After 6 cycles of R-CHOP*, the patient went into complete remission. SMZL is an indolent B cell malignancy, presents as an incidental finding or with symptoms of splenic enlargement [1,2]. Diagnosis is based on lymphocyte morphology, immunophenotype and marrow and /or splenic histology [2]. Therapeutic options include splenectomy and alkylating agents. The median survival is 10-13 years [3].