Clinical features of eleven cases with hemophagocytic syndrome presenting as acute liver failure / 中国小儿急救医学

ABSTRACT

Objective To investigate the clinical features of eleven cases of acute liver failure as the initial presentation of hemophagocytic syndrome(HPS),in order to improve the early diagnosis.Methods Eleven cases of acute liver failure as the initial presentation of HPS admitted in PICU of Shengjing Hospital affiliated to China Medical University from September 201 1 to February 2015 were investigated,the clinical manifestations,laboratory findings,therapy methods and prognosis were analyzed.Results Eleven cases of HPS had the initial symptom of acute liver failure accompanied by severe coagulation abnormalities,increase of alanine aminotransferase and aspartate aminotransferase,decrease of fibrinogen.All 1 1 cases with speno-megaly had more than 1 week thermal process.Glucocorticoid and gamma globulin were used to inhibit the activation of monocyte-macrophage cell system.Chemotherapy such as Etoposide were used as the basic treat-ment in the early stage.Plasma exchange and continuous hemodialysis and filtration were used in severe cases with bleeding tendency.One of these 11 children survived,4 cases died of multiple organ dysfunction syn-drome,and discharged six cases were followed up for mortality.Conclusion Unexplained acute liver failure, fever and cytopenias may suggest HPS,the mortality rate can be reduced by early diagnosis and treatment.