• Q Riller, F Cohen-Aubart, and D Roos-Weil.
• Service de médecine interne 2, Centre national de référence maladies systémiques rares, hôpital Pitié-Salpêtrière, Sorbonne université, Assistance publique-Hôpitaux de Paris, 75013 Paris, France. Electronic address: quentin.riller@aphp.fr.
• Rev Med Interne. 2022 Oct 1; 43 (10): 608-616.

AbstractSome common clinical situations, such as splenomegaly or lymphocytosis, or less common, such as autoimmune hemolytic anemia, cold agglutinin disease, or cryoglobulinemia can lead to the diagnosis of splenic lymphoma. Splenic lymphoma is rare, mainly of non-hodgkinian origin, encompassing very different hematological entities in their clinical and biological presentation from an aggressive form such as hepato-splenic lymphoma to indolent B-cell lymphoma not requiring treatment such as marginal zone lymphoma, the most frequent form of splenic lymphoma. These entities can be challenging to diagnose and differentiate. This review presents different clinical and biological manifestations suspicious of splenic lymphoma and proposes a diagnosis work-up. We extended the strict definition of splenic lymphoma (lymphoma exclusively involving the spleen) to lymphoma thant can be revealed by a splenomegaly and we discuss the differential diagnosis of splenomegaly.Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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