Tumor immune surveillance defect of X-linked severe combined immunodeficiency is not Epstein-Barr virus specific

Peter Mustillo, Rajinder P.S. Bajvva, Amanda M. Termuhlen, Kathleen Nicol, Rebecca Scherzer, Ronald Jaffe, Alexandra H. Filipovich, Thomas G. Gross

Research output: Contribution to journalArticlepeer-review

6 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)706-709
Number of pages4
JournalPediatric Blood and Cancer
Volume51
Issue number5
DOIs
StatePublished - Nov 1 2008
Externally publishedYes

Keywords

  • EBV
  • SCID
  • Tumor immune surveillance

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