• Piotr Buda, Piotr Gietka, Janusz B Książyk, and Maciej Machaczka.
• Department of Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.
• Arch Med Sci. 2018 Jan 1; 14 (1): 138-150.

IntroductionSecondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening syndrome of severe hyperinflammation which is often triggered by infection or autoimmune disease (macrophage activation syndrome - MAS). The aim of our study was to assess the frequency of sHLH/MAS in children treated in our institution and to compare the effectiveness of various therapeutic interventions.Material And MethodsBetween 2005 and 2013, 24 children (age: 1-17 years) were consecutively treated for sHLH/MAS. Therapy was based on glucocorticoids (GCs) in high or standard doses (hd-GCs or sd-GCs), intravenous immunoglobulin (IVIG), and cyclosporin A (CyA). A comparison of selected laboratory and clinical parameters during the first 72 h of treatment and after a week from the last intervention applied in the first 72 h after diagnosis was performed retrospectively.ResultsThe majority of patients (14/24, 58%) suffered from sHLH/MAS in the course of an autoimmune disease (12 patients diagnosed with a systemic form of juvenile idiopathic arthritis). We found with a confidence level of 95% that the application of hd-GCs in the first 24 h caused rapid alleviation of fever, reduction of hepatosplenomegaly, and an increase in thrombocytes and s-fibrinogen concentrations. The use of combination therapy with hd-GCs, IVIG, and CyA in the first 72 h caused a faster increase in s-fibrinogen. All patients survived and were alive at the follow-up of 1-8 years.ConclusionsThe results indicate that treatment of sHLH/MAS based on hd-GCs, CyA and IVIG is an effective therapy in children.

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