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- Title
Mediastinal and Disseminated Mycobacterium kansasii Disease in GATA2 Deficiency.
- Authors
Lovell, Jana P.; Zerbe, Christa S.; Olivier, Kenneth N.; Claypool, Reginald J.; Frein, Cathleen; Anderson, Victoria L.; Freeman, Alexandra F.; Holland, Steven M.
- Abstract
<bold>Rationale: </bold>Mycobacterium kansasii usually causes chronic pulmonary infections in immunocompetent patients. In contrast, disseminated M. kansasii disease is commonly associated with advanced human immunodeficiency virus infection, but is reported infrequently in other immunocompromised patients.<bold>Objectives: </bold>To identify common clinical manifestations and potential risk factors for M. kansasii infection in patients with GATA2 deficiency.<bold>Methods: </bold>We reviewed M. kansasii disease associated with GATA2 deficiency at one institution and disease associated with primary and other immunodeficiencies reported in the literature.<bold>Measurements and Main Results: </bold>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 natural killer cells. Other conditions associated with disseminated M. kansasii disease were thymic disorders and IFN-γ/IL-12 defects.<bold>Conclusions: </bold>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 indicating a need to evaluate for immunodeficiency syndromes.
- Subjects
LUNG microbiology; MYCOBACTERIAL disease diagnosis; ANTITUBERCULAR agents; GRAM-positive bacteria; INTERLEUKINS; LYMPH nodes; MEDIASTINUM; GENETIC mutation; MYCOBACTERIAL diseases; PROTEINS; RESEARCH funding; RETROSPECTIVE studies; IMMUNOCOMPROMISED patients
- Publication
Annals of the American Thoracic Society, 2016, Vol 13, Issue 12, p2169
- ISSN
2329-6933
- Publication type
journal article
- DOI
10.1513/AnnalsATS.201603-207BC