Epstein Barr virus induced lymphoma in a child with IPEX syndrome.
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.
Lucas
Kenneth G
KG
Division of Hematology/Oncology, Stem Cell Transplantation Program, Department of Pediatrics, Penn State Children's Hospital, 500 University Drive, C7830, MC H085, Hershey, Pennsylvania 17033, USA. klucas@psu.edu
Ungar
David
D
Comito
Melanie
M
Groh
Brandt
B
D002648:Child; D020031:Epstein-Barr Virus Infections / Q000188:drug therapy / Q000821:virology*; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000473:pathology / Q000628:therapy; D004854:Herpesvirus 4, Human / Q000472:pathogenicity*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000473:pathology / Q000628:therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008223:Lymphoma / Q000188:drug therapy / Q000821:virology*; D008297:Male; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications* / Q000473:pathology / Q000628:therapy; D011504:Protein-Losing Enteropathies / Q000150:complications* / Q000473:pathology / Q000628:therapy; D020123:Sirolimus / Q000627:therapeutic use; D013577:Syndrome