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Association of GATA2 Deficiency With Severe Primary Epstein-Barr Virus (EBV) Infection and EBV-associated Cancers.
- Source :
-
Clinical infectious diseases : an official publication of the Infectious Diseases Society of America [Clin Infect Dis] 2016 Jul 01; Vol. 63 (1), pp. 41-7. Date of Electronic Publication: 2016 May 11. - Publication Year :
- 2016
-
Abstract
- Background: Most patients infected with Epstein-Barr virus (EBV) are asymptomatic, have nonspecific symptoms, or have self-limiting infectious mononucleosis. EBV, however, may result in severe primary disease or cancer.<br />Methods: We report EBV diseases associated with GATA2 deficiency at one institution and describe the hematology, virology, and cytokine findings.<br />Results: Seven patients with GATA2 deficiency developed severe EBV disease. Three presented with EBV infectious mononucleosis requiring hospitalization, 1 had chronic active EBV disease (B-cell type), 1 had EBV-associated hydroa vacciniforme-like lymphoma with hemophagocytic lymphohistiocytosis, and 2 had EBV-positive smooth muscle tumors. Four of the 7 patients had severe warts and 3 had disseminated nontuberculous mycobacterial infections. All of the patients had low numbers of monocytes, B cells, CD4 T cells, and natural killer cells. All had elevated levels of EBV in the blood; 2 of 3 patients tested had expression of the EBV major immediate-early gene in the blood indicative of active EBV lytic infection. Mean plasma levels of tumor necrosis factor α, interferon γ, and interferon gamma-induced protein 10 were higher in patients with GATA2 deficiency than in controls.<br />Conclusions: GATA2 is the first gene associated with EBV hydroa vacciniforme-like lymphoma. GATA2 deficiency should be considered in patients with severe primary EBV infection or EBV-associated cancer, especially in those with disseminated nontuberculous mycobacterial disease and warts.<br /> (Published by Oxford University Press for the Infectious Diseases Society of America 2016. This work is written by (a) US Government employee(s) and is in the public domain in the US.)
- Subjects :
- Adult
Child
Cytokines blood
DNA, Viral blood
Female
GATA2 Transcription Factor genetics
Humans
Hydroa Vacciniforme
Male
Young Adult
Epstein-Barr Virus Infections complications
Epstein-Barr Virus Infections genetics
Epstein-Barr Virus Infections pathology
Epstein-Barr Virus Infections virology
GATA2 Transcription Factor deficiency
Herpesvirus 4, Human genetics
Skin Neoplasms complications
Skin Neoplasms genetics
Skin Neoplasms pathology
Skin Neoplasms virology
Subjects
Details
- Language :
- English
- ISSN :
- 1537-6591
- Volume :
- 63
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Clinical infectious diseases : an official publication of the Infectious Diseases Society of America
- Publication Type :
- Academic Journal
- Accession number :
- 27169477
- Full Text :
- https://doi.org/10.1093/cid/ciw160