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Review
. 2017 Jan 17;15(1):5.
doi: 10.1186/s12969-016-0130-4.

Macrophage Activation Syndrome: different mechanisms leading to a one clinical syndrome

Claudia Bracaglia  1 , Giusi Prencipe  2 , Fabrizio De Benedetti  2
Affiliations
Review

Macrophage Activation Syndrome: different mechanisms leading to a one clinical syndrome

Claudia Bracaglia et al. Pediatr Rheumatol Online J. .

Abstract

Background: Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages.

Main content: MAS is currently classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH). The reason is that MAS shares clinical and laboratory features with primary genetic HLH (pHLH). In this context is conceivable that some of the pathogenic mechanisms of pHLH may be involved in other forms of HLH. Heterozygosity for mutations of genes involved in pHLH may lead to a cytotoxic defect and to a development of clinical overt disease. But other different contributors might be involved to the development of MAS such as infections or underlying inflammation. In MAS, the inflammatory status of the patient is a major contributor of the disease. Indeed, the majority of the MAS episodes occurs during active disease phases or at disease onset. In addition, recent evidence in animals and humans suggest that genetics may also play a major role in contributing to hyperinflammation and particularly to macrophages hyper-responses.

Conclusions: We hypothesize that HLH may be one unique clinical syndrome, to whose generation different mechanisms may contribute, and maintained by one final effector mechanism.

Keywords: Haemophagocytic lymphohistiocytosis; IFNγ; IL-18; IL-6; Inflammasome; Macrophage activation syndrome; Natural killer.

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References

    1. Minoia F, Davi S, Horne A, Demirkaya E, Bovis F, Li C, et al. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol. 2014;66(11):3160–3169. doi: 10.1002/art.38802. - DOI - PubMed
    1. Hashemi-Sadraei N, Vejpongsa P, Baljevic M, Chen L, Idowu M. Epstein-Barr virus-related hemophagocytic lymphohistiocytosis: hematologic emergency in the critical care setting. Case Rep Hematol. 2015;2015:491567. - PMC - PubMed
    1. Kilani B, Ammari L, Kanoun F, Ben Chaabane T, Abdellatif S, Chaker E. Hemophagocytic syndrome associated with visceral leishmaniasis. Int J Infect Dis. 2006;10(1):85–86. doi: 10.1016/j.ijid.2005.03.003. - DOI - PubMed
    1. Ozdemir H, Ciftci E, Ince EU, Ertem M, Ince E, Dogru U. Hemophagocytic lymphohistiocytosis associated with 2009 pandemic influenza A (H1N1) virus infection. J Pediatr Hematol Oncol. 2011;33(2):135–137. doi: 10.1097/MPH.0b013e3181f46baf. - DOI - PubMed
    1. Singh G, Shabani-Rad MT, Vanderkooi OG, Vayalumkal JV, Kuhn SM, Guilcher GM, et al. Leishmania in HLH: a rare finding with significant treatment implications. J Pediatr Hematol Oncol. 2013;35(3):e127–e129. doi: 10.1097/MPH.0b013e318286d619. - DOI - PubMed

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