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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<jats:sup>+<\/jats:sup>CD4<jats:sup>\u2212<\/jats:sup>CD8<jats:sup>\u2212<\/jats:sup> 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\u20131022 \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/pbc.22445","type":"journal-article","created":{"date-parts":[[2010,2,16]],"date-time":"2010-02-16T16:44:35Z","timestamp":1266338675000},"page":"1020-1022","source":"Crossref","is-referenced-by-count":0,"title":["Accessory spleen: Differential diagnosis for lymphoma in autoimmune lymphoproliferative syndrome"],"prefix":"10.1002","volume":"54","author":[{"given":"Sophie","family":"Georgin\u2010Lavialle","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Achille","family":"Aouba","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Danielle","family":"Canioni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Rieux\u2010Laucat","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alain","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Hermine","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,2,16]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.cdd.4401190"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa040036"},{"key":"e_1_2_7_4_2","first-page":"18","article-title":"Inherited and acquired death receptor defects in human autoimmune lymphoproliferative syndrome","volume":"9","author":"Rieux\u2010Laucat F","year":"2006","journal-title":"Curr Dir Autoimmun"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.7539157"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V98.1.194"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/32.2.165"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198104303041822"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02749.x"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1080\/10428190310001593166"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21965"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.4.1492"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)65742-2"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004649900607"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjsurg.2007.02.002"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01655277"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00464-002-4222-5"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1002\/bjs.1800680616"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.22445","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.22445","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.22445","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,19]],"date-time":"2023-10-19T00:08:22Z","timestamp":1697674102000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.22445"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,2,16]]},"references-count":17,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2010,7]]}},"alternative-id":["10.1002\/pbc.22445"],"URL":"https:\/\/doi.org\/10.1002\/pbc.22445","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,2,16]]}}}