Rheumatologic manifestations of the "MonoMAC" syndrome. a systematic review

被引：6

作者：

Johnson, Jennifer A. ^{[1
,2
]}

Yu, Steven S. ^{[2
]}

Elist, Michael ^{[1
,2
]}

Arkfeld, Daniel ^{[1
,2
]}

Panush, Richard S. ^{[1
,2
]}

机构：

[1] Univ So Calif, Keck Sch Med, Dept Med, Div Rheumatol, Los Angeles, CA 90033 USA

[2] LAC USC Med Ctr, Los Angeles, CA USA

来源：

CLINICAL RHEUMATOLOGY | 2015年 / 34卷 / 09期

关键词：

GATA2; mutation; Monocytopenia; Mycobacterium avium complex; Panniculitis; Pseudovasculitis; Rheumatologic manifestations; Systematic review; Vasculitis; NK LYMPHOID DEFICIENCY; DENDRITIC CELL; SPORADIC MONOCYTOPENIA; AUTOSOMAL-DOMINANT; MYELODYSPLASIA; MUTATION;

D O I：

10.1007/s10067-015-2905-2

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：1643 / 1645

页数：3

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