AI Article Synopsis
- MonoMAC syndrome, first identified in 2011, is a rare condition involving low monocytosis that makes individuals more vulnerable to various infections, particularly from nontuberculous mycobacteria.
- Caused by mutations in the GATA2 gene, the syndrome can also lead to a range of systemic infections, including fungal and viral diseases.
- Recent advancements in treatment, such as haploidentical allogeneic stem cell transplants, show promise for improving survival rates, and rheumatologists might increasingly encounter patients with related rheumatologic symptoms like erythema nodosum and arthralgias.
Article Abstract
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.
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| http://dx.doi.org/10.1007/s10067-015-2905-2 | DOI Listing |
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