Von Willebrand disease: A clinical and laboratory study of sixty-five patients

Yeu Chin Chen, Luke Yang, Shin Nan Cheng, Shu Hsia Hu, Tsu Yi Chao

研究成果: 雜誌貢獻文章

10 引文 (Scopus)

摘要

von Willebrand disease (VWD) is the commonest inherited bleeding disorder, yet it has not beenwell recognized in Southeast Asia. The aim of this prospective study was to report our experience of VWD diagnosis and to establish the clinical presentations of VWD in Taiwan. From October 2003 to April 2010, 863 patients with suspicion of having an inherited bleeding disorder underwent VWD screening tests. Those with positive tests were selected for further clinical and laboratory evaluation. A nested gender- and age-matched control cohort underwent similar investigation for comparison. VWD was diagnosed by comprehensive laboratory tests including factor VIII clotting activity, von Willebrand factor antigen assay, VWF: ristocetin cofactor activity (VWF:RCo) and platelet function analyzer (PFA)-100 closure times. VWF multimer analysis was performed by western blot for disease subtype identification. Sixty-five (7.5%) patients from 55 unrelated families were discovered to have VWD. Their median age was 27 years with a range of 4 to 69 years. The most common and specific bleeding symptom in male and female patients was bleeding after dental extraction and menorrhagia, respectively, as compared with control subjects. PFA-100 epinephrine closure time was the most sensitive laboratory test for VWD diagnosis with a sensitivity of 85%, followed by VWF:RCo assay (73%). Among 49 patients with VWF multimer analysis, 37(75.5%) were revealed to have type 1 VWD. Our study demonstrates that VWD and its clinical manifestations and subtypes in Taiwan are similar to those in the West and represents the first report of its kind in a Southeast Asian population.

原文英語
頁(從 - 到)1183-1190
頁數8
期刊Annals of Hematology
90
發行號10
DOIs
出版狀態已發佈 - 十月 2011

指紋

von Willebrand Diseases
von Willebrand Factor
Hemorrhage
Taiwan
Blood Platelets
Type 1 von Willebrand Disease
Menorrhagia
Tooth Extraction
Southeastern Asia
Clinical Studies
Factor VIII
Epinephrine
Western Blotting
Prospective Studies

ASJC Scopus subject areas

  • Hematology

引用此文

Von Willebrand disease : A clinical and laboratory study of sixty-five patients. / Chen, Yeu Chin; Yang, Luke; Cheng, Shin Nan; Hu, Shu Hsia; Chao, Tsu Yi.

於: Annals of Hematology, 卷 90, 編號 10, 10.2011, p. 1183-1190.

研究成果: 雜誌貢獻文章

Chen, Yeu Chin ; Yang, Luke ; Cheng, Shin Nan ; Hu, Shu Hsia ; Chao, Tsu Yi. / Von Willebrand disease : A clinical and laboratory study of sixty-five patients. 於: Annals of Hematology. 2011 ; 卷 90, 編號 10. 頁 1183-1190.
@article{e6b76a582e7d454ab1fabacd8e50b3c2,
title = "Von Willebrand disease: A clinical and laboratory study of sixty-five patients",
abstract = "von Willebrand disease (VWD) is the commonest inherited bleeding disorder, yet it has not beenwell recognized in Southeast Asia. The aim of this prospective study was to report our experience of VWD diagnosis and to establish the clinical presentations of VWD in Taiwan. From October 2003 to April 2010, 863 patients with suspicion of having an inherited bleeding disorder underwent VWD screening tests. Those with positive tests were selected for further clinical and laboratory evaluation. A nested gender- and age-matched control cohort underwent similar investigation for comparison. VWD was diagnosed by comprehensive laboratory tests including factor VIII clotting activity, von Willebrand factor antigen assay, VWF: ristocetin cofactor activity (VWF:RCo) and platelet function analyzer (PFA)-100 closure times. VWF multimer analysis was performed by western blot for disease subtype identification. Sixty-five (7.5{\%}) patients from 55 unrelated families were discovered to have VWD. Their median age was 27 years with a range of 4 to 69 years. The most common and specific bleeding symptom in male and female patients was bleeding after dental extraction and menorrhagia, respectively, as compared with control subjects. PFA-100 epinephrine closure time was the most sensitive laboratory test for VWD diagnosis with a sensitivity of 85{\%}, followed by VWF:RCo assay (73{\%}). Among 49 patients with VWF multimer analysis, 37(75.5{\%}) were revealed to have type 1 VWD. Our study demonstrates that VWD and its clinical manifestations and subtypes in Taiwan are similar to those in the West and represents the first report of its kind in a Southeast Asian population.",
keywords = "Excessive bleeding after dental extraction, Menorrhagia, PFA-100 closure time, Ristocetin cofactor activity, Von Willebrand disease",
author = "Chen, {Yeu Chin} and Luke Yang and Cheng, {Shin Nan} and Hu, {Shu Hsia} and Chao, {Tsu Yi}",
year = "2011",
month = "10",
doi = "10.1007/s00277-011-1266-4",
language = "English",
volume = "90",
pages = "1183--1190",
journal = "Annals of Hematology",
issn = "0939-5555",
publisher = "Springer Verlag",
number = "10",

}

TY - JOUR

T1 - Von Willebrand disease

T2 - A clinical and laboratory study of sixty-five patients

AU - Chen, Yeu Chin

AU - Yang, Luke

AU - Cheng, Shin Nan

AU - Hu, Shu Hsia

AU - Chao, Tsu Yi

PY - 2011/10

Y1 - 2011/10

N2 - von Willebrand disease (VWD) is the commonest inherited bleeding disorder, yet it has not beenwell recognized in Southeast Asia. The aim of this prospective study was to report our experience of VWD diagnosis and to establish the clinical presentations of VWD in Taiwan. From October 2003 to April 2010, 863 patients with suspicion of having an inherited bleeding disorder underwent VWD screening tests. Those with positive tests were selected for further clinical and laboratory evaluation. A nested gender- and age-matched control cohort underwent similar investigation for comparison. VWD was diagnosed by comprehensive laboratory tests including factor VIII clotting activity, von Willebrand factor antigen assay, VWF: ristocetin cofactor activity (VWF:RCo) and platelet function analyzer (PFA)-100 closure times. VWF multimer analysis was performed by western blot for disease subtype identification. Sixty-five (7.5%) patients from 55 unrelated families were discovered to have VWD. Their median age was 27 years with a range of 4 to 69 years. The most common and specific bleeding symptom in male and female patients was bleeding after dental extraction and menorrhagia, respectively, as compared with control subjects. PFA-100 epinephrine closure time was the most sensitive laboratory test for VWD diagnosis with a sensitivity of 85%, followed by VWF:RCo assay (73%). Among 49 patients with VWF multimer analysis, 37(75.5%) were revealed to have type 1 VWD. Our study demonstrates that VWD and its clinical manifestations and subtypes in Taiwan are similar to those in the West and represents the first report of its kind in a Southeast Asian population.

AB - von Willebrand disease (VWD) is the commonest inherited bleeding disorder, yet it has not beenwell recognized in Southeast Asia. The aim of this prospective study was to report our experience of VWD diagnosis and to establish the clinical presentations of VWD in Taiwan. From October 2003 to April 2010, 863 patients with suspicion of having an inherited bleeding disorder underwent VWD screening tests. Those with positive tests were selected for further clinical and laboratory evaluation. A nested gender- and age-matched control cohort underwent similar investigation for comparison. VWD was diagnosed by comprehensive laboratory tests including factor VIII clotting activity, von Willebrand factor antigen assay, VWF: ristocetin cofactor activity (VWF:RCo) and platelet function analyzer (PFA)-100 closure times. VWF multimer analysis was performed by western blot for disease subtype identification. Sixty-five (7.5%) patients from 55 unrelated families were discovered to have VWD. Their median age was 27 years with a range of 4 to 69 years. The most common and specific bleeding symptom in male and female patients was bleeding after dental extraction and menorrhagia, respectively, as compared with control subjects. PFA-100 epinephrine closure time was the most sensitive laboratory test for VWD diagnosis with a sensitivity of 85%, followed by VWF:RCo assay (73%). Among 49 patients with VWF multimer analysis, 37(75.5%) were revealed to have type 1 VWD. Our study demonstrates that VWD and its clinical manifestations and subtypes in Taiwan are similar to those in the West and represents the first report of its kind in a Southeast Asian population.

KW - Excessive bleeding after dental extraction

KW - Menorrhagia

KW - PFA-100 closure time

KW - Ristocetin cofactor activity

KW - Von Willebrand disease

UR - http://www.scopus.com/inward/record.url?scp=80055066094&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=80055066094&partnerID=8YFLogxK

U2 - 10.1007/s00277-011-1266-4

DO - 10.1007/s00277-011-1266-4

M3 - Article

C2 - 21643680

AN - SCOPUS:80055066094

VL - 90

SP - 1183

EP - 1190

JO - Annals of Hematology

JF - Annals of Hematology

SN - 0939-5555

IS - 10

ER -