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von Willebrand disease: recent advances in pathophysiology and treatment
1Centeon, L.L.C., King of Prussia, Pennsylvania 19406-1310, USA. phillmd@centeon.com
Insights
This review covers new insights into von Willebrand disease (vWD) pathophysiology and treatments. It highlights vWD
Area of Science:
- Hematology
- Genetics
- Cell Biology
Background:
- Von Willebrand disease (vWD) is a common inherited bleeding disorder.
- Prevalence estimates suggest up to 1% of the population may be affected, with significant implications for women's health.
- Blood group O is associated with lower von Willebrand factor (vWF) levels.
Purpose of the Study:
- To review recent advancements in understanding the pathophysiology of vWD.
- To discuss current and emerging treatment strategies for vWD.
- To explore the latest diagnostic methods and special considerations in vWD management.
Main Methods:
- Literature review of recent studies on vWD.
- Analysis of new findings in vWF cell biology, gene regulation, and structure-function relationships.
- Discussion of diagnostic tests and therapeutic interventions.
Main Results:
- vWD is more prevalent than previously thought, impacting diverse patient groups.
- Blood group influences vWF levels, with implications for diagnosis and management.
- Advances in understanding vWF biology are informing new treatment approaches.
Conclusions:
- Updated knowledge of vWF pathophysiology is crucial for effective vWD management.
- A range of treatments exist for classical and acquired vWD, with ongoing research.
- Special populations, such as pregnant women, require tailored management strategies for vWD.
Abstract:
This review focuses on new developments in the pathophysiology and treatment of von Willebrand disease (vWd). New aspects of the cell biology, gene control, and structure-function correlates of von Willebrand factor (vWf) are reviewed. vWd is more prevalent than previously recognized, affecting up to 1% of the population; this is particularly evident in women's health. Blood group is an important determinant of von Willebrand factor levels; individuals of blood group O tend to have lower plasma levels of vWf than those in other blood groups. Currently available blood tests of vWf quantity and function are discussed, in addition to newer tests undergoing validation. Treatment of classical vWd with desmopressin acetate and plasma derivatives is discussed, as is the potential for intravenous immunoglobulin and corticosteroids in acquired vWd. Special situations, such as the management of vWd in pregnancy, are also discussed.
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