GATA2 Deficiency and Epstein–Barr Virus Disease
GATA2 is a transcription factor that binds to the promoter of hematopoietic genes. Mutations in one copy of the gene are associated with haploinsufficiency and reduced levels of protein. This results in reduced numbers of several cell types important for immune surveillance including dendritic cells...
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doaj-464b0eb578404845975415c24d342a342020-11-24T22:24:37ZengFrontiers Media S.A.Frontiers in Immunology1664-32242017-12-01810.3389/fimmu.2017.01869309962GATA2 Deficiency and Epstein–Barr Virus DiseaseJeffrey I. Cohen0Medical Virology Section, Laboratory of Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United StatesGATA2 is a transcription factor that binds to the promoter of hematopoietic genes. Mutations in one copy of the gene are associated with haploinsufficiency and reduced levels of protein. This results in reduced numbers of several cell types important for immune surveillance including dendritic cells, monocytes, CD4, and NK cells, as well as impaired NK cell function. Recently, GATA2 has been associated with several different presentations of severe Epstein–Barr virus (EBV) disease including primary infection requiring repeated hospitalizations, chronic active EBV disease, EBV-associated hydroa vacciniforme with hemophagocytosis, and EBV-positive smooth muscle tumors. EBV was found predominantly in B cells in each of the cases in which it was studied, unlike most cases of chronic active EBV disease in which the virus is usually present in T or NK cells. The variety of EBV-associated diseases seen in patients with GATA2 deficiency suggest that additional forms of severe EBV disease may be found in patients with GATA2 deficiency in the future.http://journal.frontiersin.org/article/10.3389/fimmu.2017.01869/fullGATA2Epstein–Barrchronic active Epstein–Barr virusinfectious mononucleosishydroa vacciniformesmooth muscle tumors |
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DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Jeffrey I. Cohen |
spellingShingle |
Jeffrey I. Cohen GATA2 Deficiency and Epstein–Barr Virus Disease Frontiers in Immunology GATA2 Epstein–Barr chronic active Epstein–Barr virus infectious mononucleosis hydroa vacciniforme smooth muscle tumors |
author_facet |
Jeffrey I. Cohen |
author_sort |
Jeffrey I. Cohen |
title |
GATA2 Deficiency and Epstein–Barr Virus Disease |
title_short |
GATA2 Deficiency and Epstein–Barr Virus Disease |
title_full |
GATA2 Deficiency and Epstein–Barr Virus Disease |
title_fullStr |
GATA2 Deficiency and Epstein–Barr Virus Disease |
title_full_unstemmed |
GATA2 Deficiency and Epstein–Barr Virus Disease |
title_sort |
gata2 deficiency and epstein–barr virus disease |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Immunology |
issn |
1664-3224 |
publishDate |
2017-12-01 |
description |
GATA2 is a transcription factor that binds to the promoter of hematopoietic genes. Mutations in one copy of the gene are associated with haploinsufficiency and reduced levels of protein. This results in reduced numbers of several cell types important for immune surveillance including dendritic cells, monocytes, CD4, and NK cells, as well as impaired NK cell function. Recently, GATA2 has been associated with several different presentations of severe Epstein–Barr virus (EBV) disease including primary infection requiring repeated hospitalizations, chronic active EBV disease, EBV-associated hydroa vacciniforme with hemophagocytosis, and EBV-positive smooth muscle tumors. EBV was found predominantly in B cells in each of the cases in which it was studied, unlike most cases of chronic active EBV disease in which the virus is usually present in T or NK cells. The variety of EBV-associated diseases seen in patients with GATA2 deficiency suggest that additional forms of severe EBV disease may be found in patients with GATA2 deficiency in the future. |
topic |
GATA2 Epstein–Barr chronic active Epstein–Barr virus infectious mononucleosis hydroa vacciniforme smooth muscle tumors |
url |
http://journal.frontiersin.org/article/10.3389/fimmu.2017.01869/full |
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