Contributions
Abstract: PB1815
Type: Publication Only
Background
von Willebrand disease (VWD) is the most common inherited bleeding disorder
with a prevalence of up to 1%. However in Korea, only 126 VWD patients were
registered in Korea Hemophilia Foundation (KHF).
Aims
The aim of this study was to determine
the status of VWD patients in Korea. We analyzed VWD patients by age, gender,
blood group, family history and bleeding history.
Methods
One hundred twenty-six VWD patients registered in the KHF by December
2016, and 74 patients diagnosed at six university hospitals were enrolled in this study.
We evaluated the medical records from the KHF and the questionnaires from six university
hospitals retrospectively.
Results
Seventeen patients misdiagnosed and ten patients duplicated were excluded.
One hundred nine patients registered in the KHF and 64 patients diagnosed at six university
hospitals met the criteria for VWD. The blood type O accounts for 72 (51.8%).
VWF mutation was detected in 30 patients (17.3%). Median age at diagnosis was 10.5
yr. The bleeding score of adults was higher than that of children (P＜0.001). The most
common bleeding symptom was epistaxis (48.5%). The distribution of VWD types was:
67% of type 1, 30.1% of type 2, and 2.9% of type 3.
Conclusion
Even though only six hospitals responded to the survey, 64 patients not
registered in the KHF were diagnosed with VWD. Our results suggest the prevalence
of Korean VWD might be higher than previously reported. A nationwide registration
system is warranted in order to accurately identify the national prevalence of VWD.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Von Willebrand's disease
Abstract: PB1815
Type: Publication Only
Background
von Willebrand disease (VWD) is the most common inherited bleeding disorder
with a prevalence of up to 1%. However in Korea, only 126 VWD patients were
registered in Korea Hemophilia Foundation (KHF).
Aims
The aim of this study was to determine
the status of VWD patients in Korea. We analyzed VWD patients by age, gender,
blood group, family history and bleeding history.
Methods
One hundred twenty-six VWD patients registered in the KHF by December
2016, and 74 patients diagnosed at six university hospitals were enrolled in this study.
We evaluated the medical records from the KHF and the questionnaires from six university
hospitals retrospectively.
Results
Seventeen patients misdiagnosed and ten patients duplicated were excluded.
One hundred nine patients registered in the KHF and 64 patients diagnosed at six university
hospitals met the criteria for VWD. The blood type O accounts for 72 (51.8%).
VWF mutation was detected in 30 patients (17.3%). Median age at diagnosis was 10.5
yr. The bleeding score of adults was higher than that of children (P＜0.001). The most
common bleeding symptom was epistaxis (48.5%). The distribution of VWD types was:
67% of type 1, 30.1% of type 2, and 2.9% of type 3.
Conclusion
Even though only six hospitals responded to the survey, 64 patients not
registered in the KHF were diagnosed with VWD. Our results suggest the prevalence
of Korean VWD might be higher than previously reported. A nationwide registration
system is warranted in order to accurately identify the national prevalence of VWD.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Von Willebrand's disease