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Case Reports
. 2019 Jun 24;45(1):73.
doi: 10.1186/s13052-019-0664-4.

Extreme thrombocytosis in systemic juvenile idiopathic arthritis. A case report

Alessandra Iacono  1 , Monica Sprocati  2 , Anna Lisa Giuliani  3 , Francesco Di Virgilio  3 , Caterina Borgna-Pignatti  2 , Giuseppe Maggiore  2
Affiliations
Case Reports

Extreme thrombocytosis in systemic juvenile idiopathic arthritis. A case report

Alessandra Iacono et al. Ital J Pediatr. .

Abstract

Background: Systemic onset juvenile idiopathic arthritis (SoJIA) is a rare inflammatory disorder characterized by remitting fevers, evanescent rash, generalized lymphadenopathy, hepatomegaly/splenomegaly, and/or serositis.

Case presentation: Here 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.

Conclusion: The 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.

Keywords: Case report; Systemic arthritis; Thrombocytosis.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Correlation between IL-1β and Tpo plasma levels. The graph shows the correlation between IL-1β and Tpo plasma levels at the different time points (T1, 14th day, T2, 22nd day, T3, 10 months). The correlation was not statistically significant (R = 0.887, p = 0.057)
See this image and copyright information in PMC

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