Mediastinal and Disseminated Mycobacterium kansasii Disease in GATA2 Deficiency
| Autor: | Reginald J. Claypool, Alexandra F. Freeman, Kenneth N. Olivier, Steven M. Holland, Jana P. Lovell, Cathleen Frein, Christa S. Zerbe, Victoria L. Anderson |
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| Rok vydání: | 2016 |
| Předmět: |
Adult
Male Pulmonary and Respiratory Medicine medicine.medical_specialty Mediastinal lymphadenopathy Antitubercular Agents Lymphadenopathy Mycobacterium Infections Nontuberculous Disease Immunocompromised Host Young Adult 03 medical and health sciences 0302 clinical medicine Risk Factors Internal medicine Humans Medicine In patient Disseminated disease 030212 general & internal medicine Lung Immunodeficiency Retrospective Studies Original Research Mycobacterium kansasii GATA2 Deficiency biology business.industry Potential risk Mediastinum Middle Aged equipment and supplies bacterial infections and mycoses medicine.disease biology.organism_classification Interleukin-12 GATA2 Transcription Factor 030220 oncology & carcinogenesis Mutation Immunology bacteria Female Lymph Nodes business |
| Zdroj: | Annals of the American Thoracic Society. 13:2169-2173 |
| ISSN: | 2325-6621 2329-6933 |
| Popis: | 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.To identify common clinical manifestations and potential risk factors for M. kansasii infection in patients with GATA2 deficiency.We reviewed M. kansasii disease associated with GATA2 deficiency at one institution and disease associated with primary and other immunodeficiencies reported in the literature.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, CD4Disseminated 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. |
| Databáze: | OpenAIRE |
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