Central Nervous System Involvement in Splenic Marginal Zone Lymphoma

Marginal zone B-cell lymphoma (MZL) represents a heterogeneous group of indolent non-Hodgkin lymphomas (NHL) originating from the marginal zone of B cells in lymphoid tissues. Typically, MZL is classified as nodal, extranodal, and MZL with splenic involvement. Central nervous system (CNS) involvement is rare, whether it presents as a primary dural lymphoma or as a consequence of secondary CNS involvement. CNS involvement of MZL presents with non-specific symptoms such as headaches, focal neurological deficits, cognitive impairment, and seizures in the setting of mass effect. It is essential to consider new CNS infiltration as a possibility in patients with hematological malignancies who exhibit new neurologic symptoms. Herein, we present a case of a patient with a ventriculoperitoneal shunt and recently diagnosed with splenic MZL who presented with status epilepticus and was subsequently diagnosed with secondary CNS involvement, highlighting its associated diagnostic and therapeutic challenges.