Extreme thrombocytosis in systemic juvenile idiopathic arthritis. A case report

Italian Journal of Pediatrics

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[1] 0000 0004 1757 2064, grid.8484.0, Department of Medical Sciences, Pediatrics, University of Ferrara, Ferrara, Italy;0000 0004 1757 2064, grid.8484.0, Department of Morphology, Surgery and Experimental Medicine, Section of Pathology, Oncology and Experimental Medicine, University of Ferrara, 44121 Cona, Ferrara, Italy;

关键词: Systemic arthritis;    Thrombocytosis;    Case report;   

DOI  :  10.1186/s13052-019-0664-4

来源: publisher

【 摘 要 】

BackgroundSystemic onset juvenile idiopathic arthritis (SoJIA) is a rare inflammatory disorder characterized by remitting fevers, evanescent rash, generalized lymphadenopathy, hepatomegaly/splenomegaly, and/or serositis.Case presentationHere we report the case of a 5 years-old girl with SoJIA complicated by severe thrombocytosis. Treatment with the Interleukin-1β (IL-1β) receptor antagonist Anakinra caused a fast reduction of blood platelets and of the associated systemic inflammatory response. Measurement of IL-1β, IL-6 and Tpo plasma levels at different time points confirmed the etiopathogenetic role of IL-1β in causing the thrombocytosis, while Tpo did not appear to be involved and this explains the excellent response to treatment with Anakinra.ConclusionThe excellent response to treatment with the IL-1β receptor antagonist, suggests a key pathogenic role of IL-1β in thrombocytosis as well as in the associated systemic symptoms of inflammation.

【 授权许可】

CC BY