title: [Analysis of clinical and laboratory features of 217 pediatric hemophagocytic lymphohistiocytosis].
abstract: OBJECTIVE
  To investigate the incidence, clinical symptoms, signs and laboratory features of childhood hemophagocytic lymphohistiocytosis (HLH) in China.
  
  
  METHODS
  A retrospective study was performed on 217 pediatric patients with HLH who were admitted to Children's Hospital of Chongqing Medical University from January 2006 to April 2013. All patients'medical records were reviewed and analyzed.
  
  
  RESULTS
  The Male to female ratio was 1.11:1. The median onset age was 3 years and 5 months old (range of 6 months old to 16 years and 9 months old), and the age of onset peaked between 1-5 years old (61.3%). The most common causes of HLH was infection, especially Epstein-Barr virus-associated HLH (71.0%). Other causes included malignant hemophagocytic syndrome (MAHS), macrophage activation syndrome (MAS) and so on. The outstanding clinical manifestations including persistent fever (100.0%), hepatomegaly (92.6%), splenomegaly (88.4%), and more than half of cases with central nervous system involvement and pulmonary manifestations. Laboratory data indicated that the most prominent abnormality was elevated ferritin (98.0%), and the others were hemophagocytosis in bone marrow (90.7%) and coagulation abnormalities (76.5%). Abnormal lymphocytes classification is very common in HLH.
  
  
  CONCLUSIONS
  HLH is a heterogeneous disease, with a variety of the etiology and clinical manifestations. HLH-2004 diagnostic protocol had theoretical basis and clinical operability. The hepatitis damages related indicators, lymphocytes classification, central nervous system involvement and pulmonary performance can be used as reference value for HLH diagnosis.
authors: Department of Hematology/Oncology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China.
authors: Li
authors: L
authors: Xiao
authors: Department of Hematology/Oncology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China.
authors: Xianmin
authors: X
authors: Guan
authors: Department of Hematology/Oncology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China.
authors: Yan
authors: Y
authors: Meng
authors: Department of Hematology/Oncology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China.
authors: Yongchun
authors: Y
authors: Su
authors: Department of Hematology/Oncology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China.
authors: Ying
authors: Y
authors: Xian
authors: Department of Hematology/Oncology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China.
authors: Jianwen
authors: J
authors: Xiao
authors: Department of Hematology/Oncology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China.
authors: Yinghui
authors: Y
authors: Cui
authors: Department of Hematology/Oncology, Children's Hospital, Chongqing Medical University, Chongqing 400014, China.
authors: Jie
authors: J
authors: Yu
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis*; D008297:Male; D012189:Retrospective Studies