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A study of von Willebrand's disease in Jordan
1Department of Medicine, Jordan University.
Insights
This 9-year study in Jordan found von Willebrand
Area of Science:
- Hematology
- Genetics
- Epidemiology
Background:
- Von Willebrand's disease (VWD) is an inherited bleeding disorder.
- Understanding VWD subtypes is crucial for diagnosis and management.
- Data on VWD prevalence and subtypes in Middle Eastern populations, particularly Jordan, is limited.
Purpose of the Study:
- To investigate the prevalence and subtypes of von Willebrand's disease in a Jordanian population.
- To compare the VWD subtype distribution in Jordan with findings from European and American populations.
Main Methods:
- A 9-year observational study involving 65 patients from 32 families.
- Detailed clinical and laboratory evaluation of 61 patients.
- Subtyping of VWD using von Willebrand factor multimer analysis.
Main Results:
- Von Willebrand's disease was the second most common inherited bleeding disorder after hemophilia A in the study cohort.
- Type I VWD and its variants were the most frequent, observed in 59% of patients.
- Types II B (18%) and III (11.5%) showed a higher frequency compared to European and American populations, while Type II A was observed in 11.5%.
Conclusions:
- Type I VWD is the most common subtype in Jordan.
- The observed frequencies of Type II B and Type III VWD in Jordan are higher than those reported in Western populations.
- The true prevalence of VWD in Jordan may be underestimated due to potential underdiagnosis of mild cases.
Abstract:
This work reports on the results of a 9-year study of von Willebrand's disease and its subtypes in Jordan, a country with a predominantly Arab population. There were a total of 65 patients in 32 families. Detailed study of 61 patients including von Willebrand factor multimers was done for the purpose of subtyping them. Type I and variants were seen in 36 patients (59%). Type II A and variants with decreased ristocetin response accounted for seven patients (11.5%), while 11 (18%) were of type II B. The severe type (type III) accounted for seven patients (11.5%). Von Willebrand's disease was the second most commonly seen inherited bleeding disorder after hemophilia A. It is concluded that although the observed frequency of von Willebrand's disease in this study in Jordan is lower than that in Europe and the USA, the true prevalence cannot be ascertained since many of the mild cases presumably were missed because of referral patterns. Type I and its variants was the most common type found, but the observed frequency of types II B and III was more than that reported in Europeans and Americans.
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