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: Peter Mustillo, Rajinder P S Bajwa, Amanda M Termuhlen, Kathleen Nicol, Rebecca Scherzer, Ronald Jaffe, Alexandra H Filipovich, Thomas G Gross
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 51 Issue 5 Pg. 706-9 (Nov 2008) ISSN: 1545-5017 [Electronic] United States
• PMID: 18615703 (Publication Type: Case Reports, Journal Article)
• Copyright: (c) 2008 Wiley-Liss, Inc.

Chemical References

• Antineoplastic Agents

Topics

• Antineoplastic Agents (therapeutic use)
• Bone Marrow Transplantation
• Herpesvirus 4, Human (immunology)
• Humans
• Immunologic Surveillance
• Infant
• Lymphoma, B-Cell (etiology, immunology, therapy)
• Male
• Severe Combined Immunodeficiency (complications, immunology)