title: Accessory spleen: differential diagnosis for lymphoma in autoimmune lymphoproliferative syndrome.
abstract: Mutations of Fas or, less frequently, Fas ligand genes result in a rare inherited lymphoid disorder called autoimmune lymphoproliferative syndrome (ALPS) in which lymphoma frequency is increased. We report on a patient with ALPS who had been splenectomized for giant splenomegaly and progressively developed a voluminous abdominal tumor. The histology of the removed tumor revealed that it was an accessory spleen exhibiting typical features of ALPS involvement, as shown by the presence of a large excess of CD3+CD4-CD8- T cells and plasma cells without a detectable monoclonal population. This observation highlights the lymphoma's differential diagnosis in this context.
authors.affiliation: Department of Adult Hematology, Hôpital Necker, Université Paris Descartes, Faculté de Médecine, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Georgin-Lavialle
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
authors.forename: Danielle
authors.initials: D
authors.lastname: Canioni
authors.forename: Frédéric
authors.initials: F
authors.lastname: Rieux-Laucat
authors.forename: Alain
authors.initials: A
authors.lastname: Fischer
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
mesh_terms: D000005:Abdomen / Q000473:pathology*; D000328:Adult; D056735:Autoimmune Lymphoproliferative Syndrome / Q000150:complications* / Q000235:genetics / Q000473:pathology; D002828:Choristoma / Q000473:pathology*; D003937:Diagnosis, Differential; D006801:Humans; D008223:Lymphoma / Q000473:pathology*; D008297:Male; D009154:Mutation; D013154:Spleen; D013156:Splenectomy; D019014:fas Receptor / Q000235:genetics
chemical_list: D019014:fas Receptor