title: [Clinical predictors for the phenotypic heterogeneity of severe hemophilia A in China].
abstract: OBJECTIVE
  To observe the phenotypic heterogeneity of severe hemophilia A in China and investigate the clinical factors for defining the severity of the clinical presentations.
  
  
  METHODS
  The data including the age of first bleeding and first joint bleeding, bleeding frequency, the number of joint deformities and body mass index (BMI) were collected from 223 patients with severe hemophilia A (FVIII:C≤2%).
  
  
  RESULTS
  The median age at first bleeding was 1 year (range: 0-35 years). The percentages of patients with first bleeding age<1 year, ≥2 years and ≥6 years were 44.3% (94/212), 34.4% (73/212), and 10.8% (23/212), respectively. The median age at first joint bleeding was 2.25 years. The percentages of patients with first joint bleeding age ≤1 year, ≥2 years and ≥6 years were 25.5% (24/94), 57.4% (54/94), and 18.1% (17/94), respectively. The percentage of patients who did not have joint bleeding was 7.4% (7/94). The median annual bleeding frequency was 24 per year (range: 1-120), and the proportion of patients with annual bleeding episodes of less than 6 times was 12.9%. Only 24.7% of the patients were free of any joint deformities. Analysis showed that milder cases had older ages of first bleeding and first joint bleeding than the severe cases, and the milder cases had also significantly lower BMI.
  
  
  CONCLUSIONS
  The age of first bleeding and first joint bleeding and BMI may predict the clinical severity of hemophilia A in China in the early stage.
authors.affiliation: Department of Hematology, Nanfang hospital, Southern Medical University, Guangzhou 510515, China. tomatoli@163.com
authors.forename: Han
authors.initials: H
authors.lastname: Li
authors.forename: Jing
authors.initials: J
authors.lastname: Sun
authors.forename: Xuan
authors.initials: X
authors.lastname: Zhou
authors.forename: Yang
authors.initials: Y
authors.lastname: Liu
authors.forename: Xiaoling
authors.initials: X
authors.lastname: Song
authors.forename: Qinghui
authors.initials: Q
authors.lastname: Ma
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D006467:Hemophilia A / Q000175:diagnosis / Q000453:epidemiology*; D006470:Hemorrhage; D006801:Humans; D007223:Infant; D007596:Joints / Q000002:abnormalities / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D055815:Young Adult