Tumor immune surveillance defect of X-linked severe combined immunodeficiency is not Epstein-Barr virus specific.
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.
Mustillo
Peter
P
Division of Infectious Diseases, Nationwide Children's Hospital, Columbus, Ohio, USA.
Bajwa
Rajinder P S
RP
Termuhlen
Amanda M
AM
Nicol
Kathleen
K
Scherzer
Rebecca
R
Jaffe
Ronald
R
Filipovich
Alexandra H
AH
Gross
Thomas G
TG
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*
D000970:Antineoplastic Agents