Clin Pediatr Hematol Oncol.
2017 Oct;24(2):93-100. 10.15264/cpho.2017.24.2.93.
A Multicenter Study on Korean von Willebrand Disease Realities
- Affiliations
-
- 1Department of Pediatrics, Ulsan University Hospital, Ulsan, Korea. sang@uuh.ulsan.kr
- 2Department of Pediatrics, College of Medicine, Inha University, Incheon, Korea.
- 3Korea Hemophilia Foundation, Seoul, Korea.
- 4Department of Pediatrics, Keimyung University Dongsan Medical Center, Daegu, Korea.
- 5Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Hwasun, Korea.
- 6Department of Pediatrics Kyung Hee University Hospital at Gangdong, Seoul, Korea.
- 7Department of Pediatrics, Daegu Catholic University Hospital, Daegu, Korea.
- KMID: 2396009
- DOI: http://doi.org/10.15264/cpho.2017.24.2.93
Abstract
- 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). 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.
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