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Accessory spleen: differential diagnosis for lymphoma in autoimmune lymphoproliferative syndrome. 2010
Sophie Georgin-Lavialle, and
Achille Aouba, and
Danielle Canioni, and
Frédéric Rieux-Laucat, and
Alain Fischer, and
Olivier Hermine
Department of Adult Hematology, Hôpital Necker, Université Paris Descartes, Faculté de Médecine, Assistance Publique-Hôpitaux de Paris, Paris, France.
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.
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