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Accessory spleen: Differential diagnosis for lymphoma in autoimmune lymphoproliferative syndrome
Author(s) -
GeorginLavialle Sophie,
Aouba Achille,
Canioni Danielle,
RieuxLaucat Frédéric,
Fischer Alain,
Hermine Olivier
Publication year - 2010
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22445
Subject(s) - medicine , lymphoma , lymphoproliferative disorders , differential diagnosis , context (archaeology) , pathology , spleen , monoclonal , population , cd8 , immunology , monoclonal antibody , antigen , antibody , biology , paleontology , environmental health
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. Pediatr Blood Cancer 2010;54:1020–1022 © 2010 Wiley‐Liss, Inc.