Continuous hemodialysis/hemofiltration for treatment of the critical hemophagocytic syndrome in children

Objective To study critical hemophagocytic syndrome (HPS) or macrophage activation syndrome (MAS) presented with multiple organ dysfunction syndrome (MODS) in pediatric intensive care unit (PICU),including clinical features and outcomes In order to explore the effect of bedside continuous hemodialysis/hemofiltration (CBP) as adjuvant treatment for severe HPS/MAS.Methods A total of 19 children with HPS/MAS were hospitalized met the diagnostic criteria for HPS from January,2009 to December,2012.Twelve cases were treated with CBP by continuous venin-venin hemodialysis/hemofiltration (CVVHDF) or high-volume hemofiltration (HVHF) following conventional anti-inflammatory therapy.The replacement liquid dose was 50-75 ml/ (kg · h).The organs function were evaluated and laboratory biomarkers including blood 、electrolytes,ferritin changes were measured before and after CBP treatment.Results Ninteen cases of HPS were acute onset and developed to MODS rapidiy after admission to PICU.The main clinical features were the irregular fever or high fever,hepatosplenomegaly and significant liver damage,nervous system dysfunction and disseminated intravascular coagulation (DIC).Eight cases were death and mortality rate was 42.1％,and all death occurred in those aged less than 3 years old.The mortality rate were 25％ (3/12) and 71.4％ (5/7) in CBP group and non-CBP group respectively.After CBP for 6-24 hours,the fever returned to normal range and blood electrolytes improved.The serum ferritin,serum alanine aminotransferase (ALT) and lactate dehydrogenase (LDH) reduced significantly.Serum creatinine (sCr),blood urea nitrogen (BUN) level improved.Four cases with acute respiratory distress syndrome (ARDS) improved and the ventilator parameters were downregulated.Conclusions Our findings indicate that HPS/MAS complicated with MODS is life threatening with high mortality rate.CBP therapy can lower the fever within a short time,correct electrolyte imbalance,stable circulatory function,improve the lung,liver,and brain function.It is suggested that CBP may be the potential effective therapy in severe HPS/MAS with MODS in children. Key words: Continuous blood purification;  Hemophagocytic syndrome;  Macrophage activation syndrome;  Multiple organ dysfunction syndrome;  Child