Rheumatologic manifestations of the “MonoMAC” syndrome. a systematic review

MonoMAC syndrome is characterized by monocytopenia with susceptibility to nontuberculous mycobacterial infections. First recognized in 2011, it is caused by GATA2 mutations and can manifest as disseminated mycobacterial, fungal, and viral infections. While mortality rates for this disorder have been...

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Bibliographic Details
Published in:Clinical rheumatology 2015-09, Vol.34 (9), p.1643-1645
Main Authors: Johnson, Jennifer A., Yu, Steven S., Elist, Michael, Arkfeld, Daniel, Panush, Richard S.
Format: Article
Language:English
Subjects:
Adult
Case Based Review
Erythema Nodosum - pathology
Female
GATA2 Transcription Factor - genetics
Humans
Immunologic Deficiency Syndromes - genetics
Medicine
Medicine & Public Health
Mutation
Panniculitis - pathology
Rheumatology
Young Adult
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Description
Summary:MonoMAC syndrome is characterized by monocytopenia with susceptibility to nontuberculous mycobacterial infections. First recognized in 2011, it is caused by GATA2 mutations and can manifest as disseminated mycobacterial, fungal, and viral infections. While mortality rates for this disorder have been high, it has recently been successfully treated with haploidentical allogeneic stem cell transplant. Since approximately one third of patients may have rheumatologic symptoms, such as erythema nodosum, panniculitis, or arthralgias, rheumatologists may expect to encounter this newly described entity with increasing frequency.
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-015-2905-2