BACKGROUND: The clinical characteristics of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) are largely unreported in the pediatric patients in mainland of China. The main aim of this study was to recognize the clinical features of EBV-HLH in children and to explore its prognosis and risk factors. METHODS: A retrospective study was performed on 78 pediatric patients with EBV-HLH who were admitted to Beijing Children's Hospital between 2003 and 2008. All patients' medical records were reviewed and analyzed. For each patient, demographic, clinical, laboratory and outcome information was collected. Statistical analysis was conducted via multivariate and univariate analysis. RESULTS: The age of onset peaked between 1 - 2 years and boys were more likely developed EBV-HLH. EBV-HLH occurred mainly in the serological pattern with EBV nuclear antigen (EBNA) positive (70.5%). The overall fatality of the disease was 56.7%. Twelve of the 39 fatalities (30.8%) died rapidly within 2 months after diagnosis. Multivariate analysis revealed that not receiving chemotherapy (P = 0.002), > or = 4 weeks of illness prior to diagnosis (P = 0.004), and albumin levels < 20 g/L (P = 0.045) significantly predicted an increased fatality risk. CONCLUSIONS: EBV-HLH is a severe disease with a high fatality rate that occurs mainly in the serological pattern with EBNA positive. Early initiation of chemotherapy and timely diagnosis significantly improves survival rate. Practical strategies should focus on reducing the likelihood of early death.
BACKGROUND: The clinical characteristics of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) are largely unreported in the pediatric patients in mainland of China. The main aim of this study was to recognize the clinical features of EBV-HLH in children and to explore its prognosis and risk factors. METHODS: A retrospective study was performed on 78 pediatric patients with EBV-HLH who were admitted to Beijing Children's Hospital between 2003 and 2008. All patients' medical records were reviewed and analyzed. For each patient, demographic, clinical, laboratory and outcome information was collected. Statistical analysis was conducted via multivariate and univariate analysis. RESULTS: The age of onset peaked between 1 - 2 years and boys were more likely developed EBV-HLH. EBV-HLH occurred mainly in the serological pattern with EBV nuclear antigen (EBNA) positive (70.5%). The overall fatality of the disease was 56.7%. Twelve of the 39 fatalities (30.8%) died rapidly within 2 months after diagnosis. Multivariate analysis revealed that not receiving chemotherapy (P = 0.002), > or = 4 weeks of illness prior to diagnosis (P = 0.004), and albumin levels < 20 g/L (P = 0.045) significantly predicted an increased fatality risk. CONCLUSIONS: EBV-HLH is a severe disease with a high fatality rate that occurs mainly in the serological pattern with EBNA positive. Early initiation of chemotherapy and timely diagnosis significantly improves survival rate. Practical strategies should focus on reducing the likelihood of early death.
Authors: Daniela Guimarães Rocha Ferreira; Paulo do Val Rezende; Mitiko Murao; Marcos Borato Viana; Benigna Maria de Oliveira Journal: Rev Bras Hematol Hemoter Date: 2014-07-17
Authors: Pilar Brito-Zerón; Belchin Kostov; Pedro Moral-Moral; Aleida Martínez-Zapico; Carmen Díaz-Pedroche; Guadalupe Fraile; Patricia Pérez-Guerrero; Eva Fonseca; Angel Robles; María P Vaquero-Herrero; María Andrés Calvo; María José Forner; Cesar Morcillo; José Larrañaga; Monica Rodriguez-Carballeira; Manuel Ruiz-Muñoz; Robert Hurtado-García; Sergio Prieto-González; Asun Aljibe Aguilar; Luis Caminal-Montero; Pilar Hernández-Jiménez; Cristina Rodríguez Fernández-Viagas; Pedro Castro; Victoria Morell Massó; Alejandra Flores-Chavez; Manuel Ramos-Casals Journal: Mayo Clin Proc Innov Qual Outcomes Date: 2018-08-01