angiohemophilia
Học thuậtThân thiện
Definition
Noun: A rare, inherited blood clotting disorder, also known as von Willebrand disease. It is characterized by a deficiency or defect in von Willebrand factor, a protein crucial for platelet adhesion and stabilizing factor VIII. This leads to impaired blood clotting and a tendency for prolonged bleeding, particularly from mucous membranes.
Usage Examples
- The patient was diagnosed with angiohemophilia, explaining her history of frequent nosebleeds and easy bruising.
- Management of angiohemophilia often involves medications to increase von Willebrand factor levels or promote clotting.
- Unlike classic hemophilia, angiohemophilia affects both males and females with relatively equal frequency.
Advanced Usage
- Medical Terminology: In clinical and genetic contexts, "angiohemophilia" is a synonym for von Willebrand disease (VWD). It is often specified by type (e.g., Type 1, 2, or 3) based on the quantitative or qualitative defect in the von Willebrand factor.
- Historical Context: The term is derived from the work of Finnish physician Erik von Willebrand, who first described the disorder. It combines "angio-" (relating to blood vessels), "hemo-" (blood), and "-philia" (tendency), literally meaning a "tendency for bleeding from blood vessels."
Variants and Related Words
- Von Willebrand Disease (VWD): The more common contemporary term for this condition.
- Pseudohemophilia: An older, now less common synonym for angiohemophilia, highlighting its symptomatic similarity to hemophilia.
- Hemophilia: A related group of hereditary bleeding disorders caused by deficiencies in other clotting factors (most commonly factor VIII or IX).
Synonyms
- Von Willebrand disease (VWD)
- Vascular hemophilia (historical/less common)
- Pseudohemophilia (historical/less common)
Related Phrases & Terms
- Mucosal bleeding: A hallmark symptom of angiohemophilia, referring to bleeding from the lining of areas such as the nose, gums, and gastrointestinal tract.
- Prolonged bleeding time: A key diagnostic indicator for angiohemophilia, measuring how long it takes for a small cut to stop bleeding.
- Autosomal recessive/inheritance: Describes the genetic pattern of transmission for some types of this disorder.
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