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.

A randomized study of very low‐dose factor VIII prophylaxis in severe haemophilia – A success story from a resource limited country

“…The fact that it was a single‐centre study should also be considered. Similar to our study the number of severe haemophilia A patients in Chinese study were 11, although the total number of patients were 34 (28 haemophilia A and six haemophilia B) including both severe and moderate haemophilia making the study population very heterogeneous. Second major limitation was significantly higher median age and median weight in episodic group compared to prophylaxis group.…”

Management of Hemophilia in Older Patients

“…The impact of comprehensive care is further exemplified by the analysis of 164 patients treated at the International Haemophilia Training Centre at Bangkok with blood and blood components from 1971 to 2000. The estimated probability of survival beyond 13 years of age of patients with severe hemophilia increased from 0.85 during the first decade of observation to 0.94 and 1 in the second and third decades (Chuansumrit et al 2004). The mortality rate fell from 30% to 14% and 5% over the three decades of observation.…”

Causes of Death Among 379 Patients With Hemophilia: A Developing Country’s Report

“…[6][7][8] Nowadays, given the improvement in coagulation concentrates, the life expectancy of individuals with hemophilia appears to be equal to that of individuals without hemophilia in developed countries. [9][10][11] Furthermore, recent findings have demonstrated that prophylaxis regimens decrease bleeding, pain, the frequency and duration of hospitalizations, disability, and quality of life in the hemophiliac population. Several groups of researchers 12,13 have hypothesized that there is an obvious link between the economic capacity of a country and the availability of the coagulation factors, diagnostic facilities, patients, care levels, and death rates due to bleeding disorders.…”