Macrophage activation syndrome: early diagnosis is key

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JUNB is a key transcriptional modulator of macrophage activation.

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Macrophage activation syndrome.

Macrophage activation syndrome (MAS) is a serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T lymphocytes and macrophages. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically similar to MAS, highlight the possible pathogenetic role of a defective function of perforin, a prot...

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Macrophage activation syndrome.

Macrophage Activation Syndrome is a rare and potentially fatal disease, to be considered in patients with a SIRS like clinical presentation. Falling ESR and hyperferritinemia in the appropriate clinical setting can be the greatest clues. We report a case in which a female admitted with fever and rash, initially had systemic inflammatory response syndrome (SIRS) like clinical presentation, but e...

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Macrophage activation syndrome.

There are no laid down criteria for the diagnosis of MAS. The diagnostic criteria laid down by Histiocyte Society to diagnose HLH are generally applied to the diagnosis of MAS.5 These consist of clinical features (fever, hepato-splenomegaly) along with laboratory features of cytopenia involving at least 2 cell lineages in the peripheral blood (Hb < 9 gm/dl, neutrophils < 1,000/mm3, and platelet...

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Macrophage Activation Syndrome

IMacrophages are important constituents of mononuclear phagocyte system, earlier known as reticulo-endothelial system. Tissue macrophages are innate immune cells with well-established roles in the primary response to pathogens, particularly abundant in capillary walls of lung (alveolar macrophages), Liver (Kupffer cells), spleen, brain, connective tissues, serous cavities (peritoneal macrophage...

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ژورنال

عنوان ژورنال: Open Access Rheumatology: Research and Reviews

سال: 2018

ISSN: 1179-156X

DOI: 10.2147/oarrr.s151013