Хвороба фон Віллебранда, тип 2А
ORPHA:166084· ICD-10 D68.0· Von Willebrand disease type 2A
Визначення(English summary)
A subtype of type 2 von Willebrand disease characterized by a bleeding disorder associated with a decrease in the affinity of the Willebrand factor (VWF) for platelets and the subendothelium caused by a deficiency of high molecular weight VWF multimers. The disease manifests as mucocutaneous bleeding (menorrhagia, epistaxis, gastrointestinal hemorrhage, etc.).
- Поширеність
- Unknown
- Успадкування
- Autosomal dominant, Autosomal recessive