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Хвороба фон Віллебранда

ORPHA:903· ICD-10 D68.0· Von Willebrand disease

Визначення(English summary)

A rare, inherited bleeding disorder characterized by defective platelet adhesion and secondary coagulation defect that manifests as abnormal bleeding of variable severity occurring either spontaneously or in association with an invasive procedure. Three main subtypes are defined based on the type of von Willebrand factor defect: partial (type 1) or total (type 3) deficiency, and qualitative/functional anomalies (type 2).

Поширеність
1-5 / 10 000
Успадкування
Autosomal dominant, Autosomal recessive
Вік початку
All ages