ABSTRACT
In China, the care of patients with a bleeding disorder is not organized into designated
centers with a national protocol. However, currently developed organizations of hematologists
and hemophilia patients are beginning to ensure better diagnosis, treatment, and care
of affected patients. The diagnosis of von Willebrand disease (VWD) in China is still
at an early stage. Misdiagnosis of the disease is difficult to avoid, and there is
a need to improve our current diagnostic strategy. Our data show that assessment of
von Willebrand factor (VWF) antigen levels is pivotal in ranking VWD clinical severity
for replacement therapy. Treatment choices for VWD in China are currently limited
to replacement therapy and antifibrinolytic drugs. Most VWD patients in China do not
need replacement therapy. New phenotypic assays and genetic testing of VWF for research
purposes have developed with results published in the Chinese medical literature in
the last few years, and many are practical and feasible for diagnostic application.
However, more efforts are needed for their widespread use for precise VWD diagnosis
in China.
KEYWORDS
v on Willebrand disease - diagnosis - treatment - China
REFERENCES
1
Favaloro EJ.
Toward a new paradigm for the identification and functional characterization of von
Willebrand disease.
Semin Thromb Hemost.
2009;
35
(1)
60-75
2
Sadler JE, Mannucci PM, Berntorp E et al..
Impact, diagnosis and treatment of von Willebrand disease.
Thromb Haemost.
2000;
84
(2)
160-174
3
Zhang L, Li H, Zhao H, Zhang X, Ji L, Yang R.
Retrospective analysis of 1312 patients with haemophilia and related disorders in
a single Chinese institute.
Haemophilia.
2003;
9
(6)
696-702
4
Chen YC, Chao TY, Cheng SN, Hu SH, Liu JY.
Prevalence of von Willebrand disease in women with iron deficiency anaemia and menorrhagia
in Taiwan.
Haemophilia.
2008;
14
(4)
768-774
5
Zhang JY, Wang YC, Yang L et al..
Retrospective analysis of 100 patients with von Willebrand disease [in Chinese].
Lin Chuang Hui Cui.
2003;
18
581-582
6
Wang YC, Zhang W, Cheng DW et al..
Clinical diagnosis of the type 2A von Willebrand disease [in Chinese].
Suzhou Yi Xue Yuan Xue Bao.
2000;
20
630-632
7
Hou LH, Yang LH, Liu XE.
Clinical studies on the phenotype of type 2A von Willebrand disease [in Chinese].
Shanxi Yi Yao Za Zhi.
2002;
31
182-183
8
Rodeghiero F, Castaman G, Tosetto A et al..
The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand
disease: an international, multicenter study.
J Thromb Haemost.
2005;
3
(12)
2619-2626
9
Favaloro EJ.
An update on the von Willebrand factor collagen binding assay: 21 years of age and
beyond adolescence but not yet a mature adult.
Semin Thromb Hemost.
2007;
33
(8)
727-744
10
Shi WY, Wang YC, Bai X et al..
Establishment and clinical application of collagen binding assay for von Willebrand
factor [in Chinese].
Zhonghua Jian Yan Xi Xue Za Zhi.
2003;
26
200-203
11
Zhou J, Jia YQ, Jiang H, Yang YM, Deng CQ.
Application of collagen-binding assay for von Willebrand disease.
Sichuan Da Xue Xue Bao Yi Xue Ban.
2004;
35
(1)
126-129
12
Wang YC, Shi WY, Zhang W et al..
The significance of collagen binding assay for v on Willebrand factor in diagnosis and classification of VWD [in Chinese].
Zhonghua Xue Ye Xue Za Zhi.
2003;
24
491-492
13
Favaloro EJ, Bonar R, Meiring M, Street A, Marsden K. RCPA QAP in Haematology .
2B or not 2B? Disparate discrimination of functional VWF discordance using different
assay panels or methodologies may lead to success or failure in the early identification
of type 2B VWD.
Thromb Haemost.
2007;
98
(2)
346-358
14
Xu HY, Zhao YM, Yu ZQ et al..
Detection of VWF ristocetin cofactor with ELISA method [in Chinese].
Zhen Duan Xue Li Lun Yu Shi Jian.
2007;
28
637-639
15
Xie F, Wang HL, Ding QL et al..
Development and clinical application of diagnostic test for von Willebrand disease
[in Chinese].
Zhonghua Jian Yan Yi Xi Xue Za Zhi.
2006;
29
804-806
16
Li Z, Wang Y, Wan H.
Detection of gene mutation and genetic analysis of a patient with type 3 von Willebrand
disease.
Zhonghua Xue Ye Xue Za Zhi.
1998;
19
(3)
122-124
17
Wang Y, Zhang J, Zhang W et al..
Study on genetic mutations of vWF in type 2A von Villebrand disease [in Chinese].
Zhonghua Yi Xue Yi Chuan Xue Chuan Xue Za Zhi.
2000;
17
229-232
18
Wang Y, Zhang J, Wan H, Zhang W, Ruan C.
Construction and transient expression of an Ala 737—> Glu mutant of VWF.
Zhonghua Xue Ye Xue Za Zhi.
2000;
21
(5)
256-259
19
Xie F, Wang H, Wang X et al..
Laboratory diagnosis and molecular characterization of seven Chinese von Willebrand's
disease families [in Chinese].
Zhen Duan Xue Li Lun Yu Shi Jian.
2006;
5
384-389
20
Zhou J, Yang Y, Jia YQ, Yang YM, Deng CQ, Jiang H.
Clinical application of linkage analysis for VWD family.
Sichuan Da Xue Xue Bao Yi Xue Ban.
2004;
35
(3)
391-394
21
Wang YC, Gu JM, Wan HY et al..
The genetic diagnosis and hereditary linkage analysis of a type 2N VWD pedigree [in
Chinese].
Zhonghua Xue Ye Xue Za Zhi.
2000;
21
102-103
Renchi YangM.D.
Professor, State Key Laboratory of Experimental Hematology, Institute of Hematology
and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical
College
288 Nanjing Road, Tianjin 30020, P. R. China
eMail: rcyang65@yahoo.com