[Information on von Willebrand disease].
The revised classification of von Willebrand disease (VWD) was approved by the International Society of Thrombosis and Haemostasis (ISTH)/SSC in 1993. It consists of three major categories : quantitative defect in type 1, qualitative defect in type 2, and complete deficiency in type 3. Type 2 has four subtypes : decreased GPIb binding with deficient larger multimer of VWF in type 2A, excessive GPIb binding in type 2B, defective GPIb binding with larger multimer in type 2M, and defective FVIII binding in type 2N. Subsequently, criteria for diagnosis of VWD is being reconsidered by the association. Therefore, we introduced the guidelines for diagnosis of VWD type 1 and type 2N in our department.
Nishino
M
M
Department of Pediatrics, Nara Prefectural Mimuro Hospital, Nara-pref.
Fujimura
Y
Y
D001324:Autoantigens / Q000097:blood; D003094:Collagen / Q000378:metabolism; D005169:Factor VIII / Q000378:metabolism; D006801:Humans; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000378:metabolism; D019038:Platelet Glycoprotein GPIb-IX Complex / Q000378:metabolism; D017354:Point Mutation; D012015:Reference Standards; D014842:von Willebrand Diseases / Q000145:classification / Q000175:diagnosis; D014841:von Willebrand Factor / Q000235:genetics / Q000276:immunology / Q000378:metabolism
D001324:Autoantigens; D019039:Platelet Glycoprotein GPIIb-IIIa Complex; D019038:Platelet Glycoprotein GPIb-IX Complex; D014841:von Willebrand Factor; D005169:Factor VIII; D003094:Collagen