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Despite marked systemic inflammation, the erythrocyte sedimentation rate (ESR) is paradoxically depressed, caused by low fibrinogen levels. The low ESR helps to distinguish the disorder from a flare of the underlying rheumatic disorder, in which case the ESR is usually elevated. A bone marrow biopsy or aspirate usually shows hemophagocytosis.
These inflammatory cytokines cannot be cleared and inflammatory mediators cause fever, cytopenias, coagulopathy, and central nervous system inflammation, which can progress to sepsis-like pathophysiology, shock, and death. The progression of macrophage activation in the context of rheumatic diseases is historically called MAS, and in the ...
Three types of Griscelli syndrome are recognised: type 1 has neurologic symptoms and mutations in MYO5A. The prognosis depends on the severity of neurologic manifestations. Type 2 has mutations in RAB27A and haemophagocytic syndrome, with abnormal T-cell and macrophage activation. This type has a grave prognosis if untreated.
Macrophages (/ ˈ m æ k r oʊ f eɪ dʒ /; abbreviated Mφ, MΦ or MP) are a type of white blood cell of the innate immune system that engulf and digest pathogens, such as cancer cells, microbes, cellular debris and foreign substances, which do not have proteins that are specific to healthy body cells on their surface.
The mononuclear phagocyte system and the monocyte macrophage system refer to two different entities, often mistakenly understood as one. [ citation needed ] " Reticuloendothelial system " is an older term for the mononuclear phagocyte system, but it is used less commonly now, as it is understood that most endothelial cells are not macrophages .
Inflammaging is thought to be caused by a loss of control over systemic inflammation resulting in chronic overstimulation of the innate immune system. Inflammaging is a significant risk factor in mortality and morbidity in aged individuals. [2] [3] [4] Inflammation is essential to protect against viral and bacterial infection, as well as ...
Inflammatory demyelinating diseases (IDDs), sometimes called Idiopathic (IIDDs) due to the unknown etiology of some of them, are a heterogenous group of demyelinating diseases - conditions that cause damage to myelin, the protective sheath of nerve fibers - that occur against the background of an acute or chronic inflammatory process.
IPEX (Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome) is a syndrome caused by a genetic mutation in the FOXP3 gene, [2] [3] [4] which encodes a major transcription factor of regulatory T cells (Tregs). Such a mutation leads to dysfunctional Tregs and, as a result, autoimmune diseases.