Analysis Of Prognostic Risk Factors In Childhood Hemophagocytic Lymphohistiocytosis

Objective:Hemophagocytic lymphohistiocytosis (HLH) also known as hemophagocytic syndrome (HPS) is a life-threatening hyperinflammatory syndrome, HLH occurs on the basis of inherited or acquired immune deficiencies. The main aim of this retrospective study was to investigate the clinical characteristics, diagnosis, treatment and outcomes of HLH in children and to explore the risk factors influencing mortality.Methods:A retrospective study was carried out on 65 pediatric patients with HLH who were admitted to our hospital between 2005 and 2013. All their medical records were reviewed and analyzed. For each patient, demographic, clinical and laboratory data and outcome information were collected.Results:Among the 65 HLH patients,41 were male and 24 female, the male/female ratio was 1.71:1, the age distribution at diagnosis ranged from 11 days to 12 years, the median age was 26 months. The underlying cause of HLH was Epstein-Barr virus infection in 35 patients (53.85%), autoimmune disorder in 2 patients (3.08%) and primary hemophagocytic lymphohistiocytosis (PHLH) in 2 patients (3.08%). In this study, HLH was clinically characterized by prolonged fever (65 patients,100%), hepatomegaly (63 patients,96.92%), splenomegaly (54 patients,83.08%), lymphadenopathy (48 patients,73.85%), hydrops of serous cavity (39 patients, 60.00%), skin rash (25 patients,38.46%), hemorrhage (17 patients,26.15%), jaundice (15 patients,23.08%), central nervous system involvement (11 patients,16.92%). Laboratory data indicated that liver dysfunction was the most prominent feature mainly manifested with high alanine aminotransferase (>40U/L,52 patients,80.00%), high aspartate aminotransferase (>40U/L,57 patients,87.70%), high lactate dehydrogenase (>240U/L,60 patients,92.31%) and hypoalbuminemia (55 patients, 83.08%), and the others were hyperferritinemia (65 patients,100%), cytopenias affecting at least two of three lineages in the peripheral blood (55 patients,84.62%), hypofibrinogenemia (55 patients,84.62%), hypertriglyceridemia (47 patients, 72.31%), hemophagocytosis in bone marrow (39 patients,60.94%), hyperbilirubinemia (36 patients,55.38%) and renal dysfunction (8 patients,12.31%).Univariate analysis showed that the hemorrhagic tendency and level of albumin and total bilirubin were prognostic factors. Multivariate analysis revealed that hemorrhagic tendency and hypoalbuminaemia were independent prognostic factors.Conclusion:There are various underlying diseases and clinical features for HLH. HLH is a severe disease with a high fatality rate. Early diagnosis and treatment is essential. Hemorrhagic tendency and hypoalbuminaemia are the death-associated risk factors.