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Mediastinal and Disseminated Mycobacterium kansasii Disease in GATA2 Deficiency.

Mediastinal and Disseminated Mycobacterium kansasii Disease in GATA2 Deficiency.
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Lovell JP, Zerbe CS, Olivier KN, Claypool RJ, Frein C, Anderson VL, Freeman AF, Holland SM,


Lovell JP, Zerbe CS, Olivier KN, Claypool RJ, Frein C, Anderson VL, Freeman AF, Holland SM, (click to view)

Lovell JP, Zerbe CS, Olivier KN, Claypool RJ, Frein C, Anderson VL, Freeman AF, Holland SM,

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Annals of the American Thoracic Society 2016 9 8()

Abstract
RATIONALE
Mycobacterium kansasii usually causes chronic pulmonary infections in immunocompetent patients. In contrast, disseminated M. kansasii disease is commonly associated with advanced HIV infection, but is reported infrequently in other immunocompromised patients.

OBJECTIVES
To identify common clinical manifestations and potential risk factors for M. kansasii infection in patients with GATA2 deficiency.

METHODS
We reviewed M. kansasii disease associated with GATA2 deficiency at one institution and disease associated with primary and other immunodeficiencies reported in the literature.

MEASUREMENTS AND MAIN RESULTS
Nine patients with GATA2 deficiency developed M. kansasii infections. Six patients developed disseminated disease. All patients presented with significant mediastinal lymphadenopathy or abscesses. Seven patients had pulmonary risk factors, including six smokers. The majority of patients had low numbers of neutrophils, monocytes, B cells, CD4 T cells, and NK cells. Other conditions associated with disseminated M. kansasii disease were thymic disorders and IFN-γ/IL-12 defects.

CONCLUSIONS
Disseminated M. kansasii disease involving mediastinal lymph nodes is surprisingly common in GATA2 deficiency, but also occurs in defects of IFN-γ synthesis and response. Disseminated M. kansasii should be considered a marker for need to evaluate for immunodeficiency syndromes.

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