Atypical diffuse large B-cell lymphoma, primary splenic lymphoma variant; a case report

INTRODUCTION AND IMPORTANCE: Primary splenic lymphoma (PSL) is characterized by lymphoma involvement confined to the spleen and hilar lymph nodes, without evidence of liver involvement or other sites. This condition is extremely uncommon, accounting for approximately 1 % of non- Hodgkin lymphomas (NHLs) and <2 % of all lymphomas. Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of both PSLs and all NHLs. DLBCL encompasses an aggressive heterogeneous entity with distinct morphological variants. CASE PRESENTATION: A 68 year-old gentleman presented to the office with a 10-month history of vague left sided upper abdominal pain. Clinical examination revealed a tender left upper quadrant, evidenced with splenomegaly on radiological evaluation. The patient proceeded with a splenectomy with subsequent pathological and immunohistochemical analysis, confirming a final diagnosis of germinal center type DLBCL. CLINICAL DISCUSSION: Primary splenic DLBCL is a rare variant of DLBCL, characterized by exclusive involvement of the spleen. It requires a comprehensive diagnostic evaluation to exclude lymphoma involvement in other organs and lymph nodes. Splenectomy followed by appropriate adjuvant therapy has been demonstrated as the definitive treatment strategy. This case report emphasizes the importance of considering primary splenic DLBCL as a differential diagnosis in patients presenting with splenomegaly and highlights the significance of multidisciplinary collaboration for accurate diagnosis and optimal management of this uncommon entity. Conclusion: Primary Splenic DLBCL, an exceptionally rare B-Cell neoplasm variant, requires precise diagnosis due to its unique splenic involvement. Splenectomy's efficacy, adjuvant therapy, multidisciplinary collaboration, and ongoing research are crucial for optimal management.