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vol. 14
Clinical research

The influence of various therapeutic regimens on early clinical and laboratory response and outcome of children with secondary hemophagocytic lymphohistiocytosis

Piotr Buda, Piotr Gietka, Janusz B. Książyk, Maciej Machaczka

Arch Med Sci 2018; 14, 1: 138–150
Online publish date: 2016/03/04
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Introduction: Secondary 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 methods: Between 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.

Results: The 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.

Conclusions: The results indicate that treatment of sHLH/MAS based on hd-GCs, CyA and IVIG is an effective therapy in children.

hemophagocytic lymphohistiocytosis, macrophage activation syndrome, juvenile idiopathic arthritis, children

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