Abstract
BACKGROUND: Primary splenic lymphoma represents a rare entity that constitutes less than 1% of non-Hodgkin lymphomas, and less than 2% of all lymphomas. Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of primary splenic lymphomas. DLBCL encompasses a heterogeneous entity with distinct morphological variants. The anaplastic variant of DLBCL was first defined in the 2017 World Health Organization classification as a rare histological subtype that constitutes less than 3.4% of DLBCL cases. CASE PRESENTATION: A 65-year-old Syrian man presented to our hospital with constant dull localized left upper quadrant abdominal pain for about 20 days accompanied by general weakness, loss of appetite, and rapid weight loss. Clinical examination revealed isolated splenomegaly and left upper abdominal tenderness. Following physical, laboratory, and radiologic examinations, the patient underwent splenectomy. Interestingly, pathological and immunohistochemical examinations of the resected spleen confirmed the diagnosis of a primary anaplastic variant of DLBCL. CONCLUSIONS: Herein, we aimed to present an unusual combination of a rare splenic neoplasm and a unique lymphoma subtype. Furthermore, we aimed to highlight the difficulties in differential diagnosis and the importance of histological and immunohistochemical examinations with clinical correlation.