title: Tumor immune surveillance defect of X-linked severe combined immunodeficiency is not Epstein-Barr virus specific.
abstract: Immunodeficient patients are at an increased risk of developing Epstein-Barr virus (EBV) associated lymphomas. We report a patient with X-linked severe combined immunodeficiency (X-SCID), who presented with an EBV-negative, B-cell non-Hodgkin lymphoma. The tumor did not resolve with chemotherapy or rituximab, but only after recovery of functional donor T-cells cell following hematopoietic stem cell transplantation (HSCT). This case illustrates that the cancer predilection associated with immunodeficient hosts may not be a specific immune defect in the recognition of viral specific antigens, and it could be a defect in immune surveillance necessary for elimination of cells with abnormalities in proliferation, function and/or apoptosis.
authors.affiliation: Division of Infectious Diseases, Nationwide Children's Hospital, Columbus, Ohio, USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Mustillo
authors.forename: Rajinder P S
authors.initials: RP
authors.lastname: Bajwa
authors.forename: Amanda M
authors.initials: AM
authors.lastname: Termuhlen
authors.forename: Kathleen
authors.initials: K
authors.lastname: Nicol
authors.forename: Rebecca
authors.initials: R
authors.lastname: Scherzer
authors.forename: Ronald
authors.initials: R
authors.lastname: Jaffe
authors.forename: Alexandra H
authors.initials: AH
authors.lastname: Filipovich
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Gross
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D004854:Herpesvirus 4, Human / Q000276:immunology*; D006801:Humans; D007157:Immunologic Surveillance; D007223:Infant; D016393:Lymphoma, B-Cell / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000276:immunology*
chemical_list: D000970:Antineoplastic Agents