Accessory Spleen: Differential Diagnosis for Lymphoma in Autoimmune Lymphoproliferative Syndrome

作者：Georgin Lavialle Sophie; Aouba Achille; Canioni Danielle; Rieux Laucat Frederic; Fischer Alain; Hermine Olivier^*

来源：Pediatric Blood and Cancer, 2010, 54(7): 1020-1022.

DOI：10.1002/pbc.22445

• accessory spleen
• autoimmune lymphoproliferative syndrome
• FAS deficiency
• lymphoma
• splenectomy

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. Pediatr Blood Cancer 2010;54: 1020-1022.

• 出版日期2010-7-1