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

doi:10.1002/pbc.21651

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

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7 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 language	English (US)
Pages (from-to)	706-709
Number of pages	4
Journal	Pediatric Blood and Cancer
Volume	51
Issue number	5
DOIs	https://doi.org/10.1002/pbc.21651
State	Published - Nov 2008
Externally published	Yes

Keywords

EBV
SCID
Tumor immune surveillance

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This output contributes to the following UN Sustainable Development Goals (SDGs)

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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.",

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AU - Mustillo, Peter

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AU - Termuhlen, Amanda M.

AU - Nicol, Kathleen

AU - Scherzer, Rebecca

AU - Jaffe, Ronald

AU - Filipovich, Alexandra H.

AU - Gross, Thomas G.

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AB - 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.

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

Abstract

Keywords

UN SDGs

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