title: Survival analysis of patients with haemophilia at the International Haemophilia Training Centre, Bangkok, Thailand.
abstract: Haemophiliac treatment in less developed countries is limited to locally prepared fresh frozen plasma, cryoprecipitate, cryo-removed plasma and lyophilized products as replacement therapy. Factor concentrate is seldom used because of the high price. The present study reports the survival analysis of 164 patients comprising 138 haemophilia A and 26 haemophilia B cases from 134 families registered at the International Haemophilia Training Centre-Bangkok, Faculty of Medicine, Ramathibodi Hospital, Mahidol University from 1971 to 2000. The duration of follow-up ranged from 1 to 27 years and 8 months with a median of 9 years and 1 month. They received treatment on demand of bleedings, and 85 patients received additional home treatment for early bleedings. The proportion of death was 15.2%. The Kaplan-Meier survival curves revealed the median (95% CI) survival time of patients with severe and moderate degrees of 35 years and 6 months (21.4-49.7), and 38 years and 1 month (28.8-47.3), respectively. To compare the progressive achievement of haemophilia care services, the treatment period was divided into three decades: 1971-1980, 1981-1990 and 1991-2000. The patients with unaided proper walking increased from 62.8% in the first decade to 84.5% in the third decade. However, one-third of the patients developed one to four chronic haemarthrosis determined by clinical evaluation, especially patients with severe degree. Moreover, the estimated probability of a survival time beyond 13 years of age among patients with severe degree increased from 0.85 in the first decade to 0.94 and 1 in the second and third decades, respectively. Thus, established haemophilia care, even with limited resources, has significantly decreased the risk of death and increased the survival time among patients with haemophilia.
authors.affiliation: Department of Paediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. raajs@mahidol.ac.th
authors.forename: A
authors.initials: A
authors.lastname: Chuansumrit
authors.forename: S
authors.initials: S
authors.lastname: Krasaesub
authors.forename: P
authors.initials: P
authors.lastname: Angchaisuksiri
authors.forename: P
authors.initials: P
authors.lastname: Hathirat
authors.forename: P
authors.initials: P
authors.lastname: Isarangkura
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D003729:Dental Care / Q000706:statistics & numerical data; D006395:Hemarthrosis / Q000453:epidemiology; D006467:Hemophilia A / Q000401:mortality*; D006470:Hemorrhage / Q000453:epidemiology; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D008875:Middle Aged; D016019:Survival Analysis; D015996:Survival Rate; D013785:Thailand / Q000453:epidemiology; D065227:Transfusion Reaction