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

Medical and Pediatric Oncology
J I HenterO Söder

Abstract

The familial form of hemophagocytic lymphohistiocytosis (HLH) is an inherited disease with disturbed immunomodulation and characterized by fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, and coagulopathy, i.e., findings which are similar to many of the reported biological effects of the inflammatory cytokines. Due to the previously shown hypercytokinemia in active HLH with elevated levels of interleukin (IL)-6, tumor necrosis factor-alpha, and interferon-gamma, it has been suggested that cytokine dysregulation may be of pathophysiological importance. Here we have assayed the serum levels of the members of the IL-1 ligand family, the two agonists IL-1 alpha and IL-1 beta and the antagonist IL-1 receptor antagonist (IL-1ra), in nine children with HLH and cerebrospinal fluid (CSF) specimens from four children. Serum IL-1ra was elevated in all patients with active disease to a degree which correlated well with disease activity. Furthermore, the levels decreased day by day during treatment of a patient who suffered a relapse. Moreover, high levels of IL-1ra were also detected in CSF during active disease. However, IL-1 beta levels were all within normal limits and circulating IL-1 alpha levels were normal in all but two ...Continue Reading

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Citations

May 13, 2009·Pediatric Blood & Cancer·Ulf TitzeCarl Friedrich Classen
Dec 8, 2006·European Journal of Pediatrics·Gritta E Janka
Aug 13, 2010·Zeitschrift für Rheumatologie·I Kötter, G Horneff
Mar 4, 2014·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·Surender RajasekaranJames Birmingham
Mar 25, 2016·Nature Reviews. Rheumatology·Alexei A GromFabrizio De Benedetti
Dec 21, 2010·Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases·Normi BruckFrank Pessler
Nov 12, 2014·Annual Review of Medicine·Grant S Schulert, Alexei A Grom
Feb 6, 2017·Current Treatment Options in Neurology·AnnaCarin HorneGritta Janka
Dec 17, 2003·British Journal of Haematology·Gritta E Janka, E M Schneider

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