TY - JOUR JO - Archives of Medical Science SN - 1734-1922 VL - 14 IS - 1 PY - 2018 ID - Buda2018 TI - The influence of various therapeutic regimens on early clinical and laboratory response and outcome of children with secondary hemophagocytic lymphohistiocytosis AB - 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. AU - Buda, Piotr AU - Gietka, Piotr AU - Książyk, Janusz B. AU - Machaczka, Maciej SP - 138 EP - 150 DA - 2018 DO - 10.5114/aoms.2015.56325 UR - http://dx.doi.org/10.5114/aoms.2015.56325 ER -