Wandering Lymphoma: A Case of Wandering Spleen Secondary to Splenic Marginal Zone Lymphoma

Splenic marginal zone lymphoma (SMZL) is a rare subtype of non-Hodgkin lymphoma. It often follows an indolent course presenting with splenomegaly. Treatment varies from clinically monitoring to splenectomy, chemotherapy, and rituximab treatments. Wandering spleen is an even rarer condition with less than 500 cases reported in the literature, often resulting from laxity in the suspensory ligaments supporting the spleen. Wandering spleen most commonly affects women of childbearing age and children. Treatment for this can be either nonsurgical or surgical with splenopexy or splenectomy.

A 75-year-old female initially presented from Mexico for evaluation of splenomegaly and abdominal distension. Computer tomography (CT) scan of the abdomen and pelvis showed a pelvic mass, and labs showed leukocytosis with lymphocytic predominance. The pelvic mass was later confirmed as a wandering spleen. Flow cytometry on peripheral blood was diagnostic for SMZL. The patient was diagnosed with wandering spleen secondary to SMZL and treated with splenectomy.

Wandering spleen is very rare and is typically associated with pregnancy, splenomegaly, or inborn errors of the ligaments supporting the spleen. This is only the fifth case reported in the literature of wandering spleen secondary to a lymphoma and the first reported with SMZL. This case offers a simultaneous presentation of two very rare conditions presenting in an atypical fashion. SMZL commonly presents in this age group; however, wandering spleen is rarely seen at this age. Additionally, this patient's first-line treatment for her SMZL was nontraditional with only a splenectomy with no rituximab administered due to the risk of torsion and infarction of the wandering spleen.