Francesco Bertoni, MD

No definite guidelines exist for the management of nongastric MALT lymphoma. Retrospective series have included patients treated with different modalities, and excellent cause-specific and overall survival have been demonstrated, independent of the type of treatment adopted.

In this article, we review the current knowledge on the biological findings, clinical features, and therapeutic approaches for splenic marginal zone lymphoma.

The aim of this review is twofold: to summarize descriptions of the clinical presentation provided in published series in order to help clinicians recognize and treat patients, and to discuss diagnostic difficulties faced by hematopathologists when dealing with these lesions and others in the differential diagnosis that must be distinguished from one another.

Marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent B-cell non-Hodgkin lymphoma arising from the lymphoid tissue at extranodal sites.