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Epstein Barr virus induced lymphoma in a child with IPEX syndrome
Author(s) -
Lucas Kenneth G.,
Ungar David,
Comito Melanie,
Groh Brandt
Publication year - 2008
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.21341
Subject(s) - medicine , autoimmunity , enteropathy , lymphoma , immunology , immune dysregulation , immune system , disease
IPEX syndrome (immune deficiency, polyendocrinopathy, enteropathy, X‐linked) is a disorder or regulatory T cell (Treg) function which can result in early death due to infection or complications related to autoimmunity. Therapeutic options for these patients can include allogeneic stem cell transplantation (SCT) or the use of immunosuppressive regimens to control the manifestations of autoimmunity. We report a patient with IPEX syndrome who was managed with rapamycin and subsequently developed EBV induced lymphoma. Pediatr Blood Cancer 2008;50:1056–1057. © 2007 Wiley‐Liss, Inc.