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Review
. 2014 Aug;16(8):869-73.

[MonoMAC syndrome]

[Article in Chinese]
Zhao-Long Chen  1 , Yun-Fei An, Xiao-Dong Zhao
Affiliations
  • PMID: 25140787
Free article
Review

[MonoMAC syndrome]

[Article in Chinese]
Zhao-Long Chen et al. Zhongguo Dang Dai Er Ke Za Zhi. 2014 Aug.
Free article

Abstract

MonoMAC syndrome is a newly discovered immune deficiency syndrome caused by GATA-2 mutation, which is an autosomal dominant genetic disease. MonoMAC syndrome has typical immune cell abnormalities, with severe infection and is prone to develop into a hematological disease. Therapeutics for this disease mainly relies on symptomatic treatment and hematopoietic stem cell transplantation. In this paper, the research advances in clinical manifestations, laboratory tests, pathogenesis, diagnosis and treatment of MonoMAC syndrome are reviewed.

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