vascular hemophilia
Học thuậtThân thiện
Definition
Noun: 1. A hereditary bleeding disorder: A form of hemophilia characterized by a deficiency or defect in a specific blood-clotting protein (von Willebrand factor), leading to impaired platelet function and prolonged bleeding, typically from mucous membranes.
Usage
- Vascular hemophilia is the original name for what is now more commonly called von Willebrand disease.
- Diagnosis of vascular hemophilia involves specific tests for clotting factor levels.
- Unlike the more well-known hemophilia A, vascular hemophilia affects both males and females.
Examples
- The patient was diagnosed with vascular hemophilia after experiencing frequent nosebleeds and easy bruising.
- Management of vascular hemophilia often involves medication to increase the level of the deficient clotting factor.
- Research into vascular hemophilia has improved treatment options significantly.
Advanced Usage
- Medical Classification: In clinical hematology, vascular hemophilia is classified into several types (e.g., Type 1, 2, 3) based on the severity and nature of the von Willebrand factor defect.
Variants and Related Words
- von Willebrand disease (VWD): The modern and more frequently used term for this condition.
- Angiohemophilia: An older, synonymous term.
- Hemophilia: The broader category of bleeding disorders to which vascular hemophilia belongs.
Synonyms
- von Willebrand disease (VWD)
- von Willebrand's disease
- Angiohemophilia (less common)
Notes on Meaning
- The term vascular hemophilia specifically highlights the involvement of the blood vessels ('vascular') and the tendency for bleeding from mucous membranes, distinguishing it from other forms of hemophilia that primarily involve joint and muscle bleeding. Its discovery by Erik von Willebrand is a key historical detail associated with the term.
Noun
- a form of hemophilia discovered by Erik von Willebrand; a genetic disorder that is inherited as an autosomal recessive trait; characterized by a deficiency of the coagulation factor and by mucosal bleeding