Current Status and Comprehensive Care of Korean Hemophiliacs

Kim, Eun Joo; Lee, Hahng

Abstract

BACKGROUND: Hemophilia A and B are sex- linked disorders that are due to respective deficiencies of clotting factors VIII and IX. Because hemophilia is life-long and life-threatening bleeding disorder, comprehensive care is very important and a lot of medical resources are required. We analyzed the status of Korean hemophiliacs to promote the comprehensive care of hemophilia and to utilize the limited medical resources appropriately.
METHODS
We evaluated the past medical history for the 1,335 hemophilia patients who were registered in the Korea Hemophilia Foundation (KHF) from February 1991 to June 1999. Coagulation factor assay, viral studies, liver function tests and radiologic examination for orthopedic sequales were also performed.
RESULTS
Of the total patients, hemophilia A and B were 1,142 cases (85.5%) and 193 cases (14.5%), respectively. Severe cases were 892 (66.8%), moderate 344 (25.8%) and mild 90 (6.7%). Their ages ranged from 7 months to 81 years and the median age was 18 years and 11 months. The number of patients diagnosed under 1 year old was 365 (28.5%). The family history of hemophilia was revealed in 55.4% of patients. 1,144 cases (90.7%) of 1,261 hemophiliacs experienced hemarthrosis, 61.9% epistaxis, 41.2% hematuria, 32.5% gastrointestinal hemorrhage and 15.4% intracranial hemorrhage. 217 cases (25.0%) of 868 severe patients bled over four times a month. Annual usage of factor concentrates per one patient in 1998 was average 44,306 IU/year. Average yearly usage of factor concentrates per kilogram body weight was 1,018 IU/kg/ year. Home treatment program was introduced to 59.2% of patients. Chronic hemophilic arthropathies were recognized in 791 cases (69.9%) of 1,131 patients. The frequencies of inhibitor development in hemophilia A and B were 5.1% and 4.3%, respectively. The prevalence of anti-HCV positivity was 48.9%, HBsAg positivity, 3.7% and anti-HIV positivity, 1.6%.
CONCLUSION
The national registries and surveillance systems for hemophilia should be more firmly established to achieve comprehensive care of hemophilia and utilize the limited medical resources appropriately.