Elevated circulating levels of interleukin-1 receptor antagonist but not IL-1 agonists in hemophagocytic lymphohistiocytosis

Association of transforming growth factor- 1 gene polymorphism in the development of Epstein-Barr virus-related hematologic diseases

“…22 By contrast, no significant increases of serum IL-1α and β levels were found in HLH patients. 23 We previously noted that serum levels of IL-1 were high in a limited number of patients with HLH in spite of extremely high levels of IFN-γ, sIL-2R, IL-6 and TNF in many such patients.…”

Gene-expression signatures differ between different clinical forms of familial hemophagocytic lymphohistiocytosis

“…1,10 Hemophagocytosis is an indicator of cytokinedriven macrophages and histiocytes. 1,10 All genetic forms of FHL can be rapidly fatal if left untreated. 1,9,10 Affected patients die of overwhelming infections or uncontrolled systemic inflammation and multiorgan failure.…”

“…Other studies have revealed elevated plasma levels of IL-12 and IL-10. 1,10 Hemophagocytosis is an indicator of cytokinedriven macrophages and histiocytes. 1,10 All genetic forms of FHL can be rapidly fatal if left untreated.…”

Autoimmune-Associated Hemophagocytic Syndrome/Macrophage Activation Syndrome

“…MAS as a first symptom of sJIA may be indistinguishable from other cases of HLH when arthritis is missing. A high interleukin-1 concentration in blood may also suggest MAS rather than classic HLH (Janka, 2009;Henter et al, 1996). Although mild elevation of sIL-2R has been reported in many rheumatic diseases including JIA and SLE, a several-fold increase in the levels of sIL-2R in these diseases is highly suggestive of MAS (Grom & Mellins, 2010).…”

“…The immune response is often triggered by different stimulants (e.g., infection) and the underlying inherited or acquired immune defect. It has been proposed that the clinical presentation of HLH is due to uncontrolled activation of immune cells, macrophages and CD8 + T lymphocytes (cytotoxic), leading to a massive release of various mediators of inflammation such as TNF-(tumor necrosis factor ), interleukin(IL)-6, IL-8, IL-10, IL-12, IL-18, interferon , macrophage inflammatory protein (MIP 1-), and hematopoietic growth factors (e.g., GM-CSF) (Filipovich, 2009;Henter et al, 1991aHenter et al, , 1996Henter et al, , 2007Janka & Schneider, 2004;Osugi et al, 1997). IL-10 with its anti-inflammatory properties plays many important roles in the regulation of autoimmune inflammatory responses, particularly of systemic autoimmune disorders such as HLH/MAS.…”