1. Tumor immune surveillance defect of X-linked severe combined immunodeficiency is not Epstein-Barr virus specific
- Author
-
Rebecca Scherzer, Kathleen Nicol, Peter Mustillo, Rajinder P.S. Bajwa, Alexandra H. Filipovich, Ronald Jaffe, Amanda Termuhlen, and Thomas G. Gross
- Subjects
Male ,Herpesvirus 4, Human ,Lymphoma, B-Cell ,medicine.medical_treatment ,Antineoplastic Agents ,Hematopoietic stem cell transplantation ,medicine.disease_cause ,Virus ,hemic and lymphatic diseases ,medicine ,Humans ,X-linked severe combined immunodeficiency ,Immunologic Surveillance ,Bone Marrow Transplantation ,Severe combined immunodeficiency ,business.industry ,Cancer ,Infant ,Hematology ,medicine.disease ,Epstein–Barr virus ,Lymphoma ,Oncology ,Pediatrics, Perinatology and Child Health ,Immunology ,Rituximab ,Severe Combined Immunodeficiency ,business ,medicine.drug - 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. Pediatr Blood Cancer 2008;51:706–709. © 2008 Wiley-Liss, Inc.
- Published
- 2008