A pilot study to detect von willebrand disease in women with menorrhagia
Von Willebrand disease (vWD) is the most common inherited bleeding disorders, found in approximately 1% of the general population, without ethnic differences. vWD results from a qualitative or quantitative defect in von Willebrand factor (vWF) resulting in impaired primary homeostasis. Menorrhagi...
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Main Author: | |
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Format: | Thesis |
Language: | English |
Published: |
2007
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Subjects: | |
Online Access: | http://eprints.usm.my/48423/1/DR.%20%20WAN%20ASWANI%20BT.%20WAN%20YUSOF-24%20pages.pdf http://eprints.usm.my/48423/ |
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Summary: | Von Willebrand disease (vWD) is the most common inherited bleeding disorders, found
in approximately 1% of the general population, without ethnic differences. vWD results
from a qualitative or quantitative defect in von Willebrand factor (vWF) resulting in
impaired primary homeostasis. Menorrhagia is defined objectively as > 80ml menstrual
blood loss per cycle or menses lasting longer than 7 days and is a common gynecologic
problem in women of reproductive age. However, in 50% of cases, no pathology is
detectable. Menorrhagia is valuable predictors of bleeding disorder in women. The
frequency of vWD in women with menorrhagia ranges from 5-50% in different studies.
The aim of this study is to know the prevalence of vWD in women with menorrhagia of
unknown cause and identify the subtypes of the disease.
Thirty women who came to Obstetric and Gynecology Clinic, HUSM with menorrhagia
without uterine pathology was selected for this study. A detailed history related to
menorrhagia was acquired from them. Full blood count (FBC), prothrombin time (PT),
activated partial prothrombin time (APTT), ABO blood grouping, factor VIII activity
(FVIII: C), von Willebrand factor antigen (vWF: Ag) and von Willebrand factor activity
(vWF: Ac) and collagen binding assays (vWF: CBA) were measured in all patients.
Subsequently ristocetin induced platelet aggregation (RIP A) was performed for those
who had abnormal von Willebrand studies.
Out of 30, 97% were Malay with median age of 42 years old. Four (13.3%) patients have
abnormal parameters of von Willebrand studies. Two were diagnosed as 'possible' vWD
Type 1, one patient as von Wille brand deficiency related to blood group 0 and the other
one as 'possible' vWD Type 2 either subtype 2A or 2M. There is no association between
age, onset of menorrhagia, dtmrtion of menstruation, history of blood transfusion or other
bleeding tendencies with the development of the disease.
Based on von Willebrand studies, von Willebrand functional assays (vWF: Ac and vWF:
CBA) was significantly lower than vWF: Ag and FVIII: C in this 4 patients. Effect of
hormone replacement therapy and blood group were analyzed and both did not show
statistically significant in contributing to von Willebrand profiles.
In conclusion, to date this is the first reported cases of vWD among Malaysian women
with menorrhagia without uterine pathology. vWD is highly prevalence and though
coagulation screening test is not helpful, we would like to suggest that a von Willebrand
studies is directed to patient at least before planning for an invasive procedure. |
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