Association of GATA2 Deficiency With Severe Primary Epstein-Barr Virus (EBV) Infection and EBV-associated Cancers
2016
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.We report EBV diseases associated with
GATA2deficiency at one institution and describe the hematology, virology, and cytokine findings.Seven patients with
GATA2deficiency developed severe EBV disease. Three presented with EBV infectious
mononucleosisrequiring 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 genein 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
GATA2deficiency than in controls.GATA2 is the first gene associated with EBV
hydroa vacciniforme-like lymphoma.
GATA2deficiency should be considered in patients with severe primary EBV infection or EBV-associated cancer, especially in those with disseminated nontuberculous mycobacterial disease and warts.
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