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- W53789077 abstract "agents. Malignancies and especially lymphomas can be associated with reactive HPS. The prevalence of HPS is difficult to estimate due to its rarity. A special form of HPS develops in association with autoimmune diseases and is termed macrophage-activation syndrome. MAS occurs most commonly in the setting of systemic juvenile rheumatoid arthritis, systemic lupus erythematosus, adult Still's disease and Sjogren syndrome, but occurrence with other autoimmune diseases such as systemic sclerosis and vasculitis has been reported [1]. The prevalence of MAS in one report was 3% [1]. Mortality due to MAS or HPS associated with autoimmune diseases is significantly high, 38.5%, according to a recent French multicenter study [2]. Clinically, HPS is characterized by persistent fever, lymphadenopathy, hepatosplenomegaly and pancytopenia. Typical laboratory abnormalities include elevated levels of liver transminases, bilirubin, lactate dehydrogenase, ferritin and triglycerides, but low levels of fibrinogen. The active phase of the disease is characterized by increased levels of the α-chain of the soluble interleukin-2R (sCD25), which makes it a valuable marker for diagnosis [3] as well as for prognosis since increased levels correlate with increased mortality [4]. Impairment or absence of NK and cytotoxic T cell activity has also been demonstrated [5]. The clinical picture and laboratory manifestations result from increased levels of inflammatory cytokines secreted" @default.
- W53789077 created "2016-06-24" @default.
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- W53789077 date "2009-10-01" @default.
- W53789077 modified "2023-09-23" @default.
- W53789077 title "The hemophagocytic syndrome/macrophage activation syndrome: a final common pathway of a cytokine storm." @default.
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