Accessory spleen: differential diagnosis for lymphoma in autoimmune lymphoproliferative syndrome.
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.
Georgin-Lavialle
Sophie
S
Department of Adult Hematology, Hôpital Necker, Université Paris Descartes, Faculté de Médecine, Assistance Publique-Hôpitaux de Paris, Paris, France.
Aouba
Achille
A
Canioni
Danielle
D
Rieux-Laucat
Frédéric
F
Fischer
Alain
A
Hermine
Olivier
O
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
D019014:fas Receptor