von Willebrand disease: laboratory aspects of diagnosis and treatment.
von Willebrand disease is the most common inherited bleeding disorder in humans. VWD can be classified into three major types, designated Types 1, 2 and 3; Type 2 can be further separated into subtypes 2A, 2B, 2M and 2N. The diagnosis of VWD requires a personal and family history of bleeding and confirmation by laboratory analysis. Although Types 2 and 3 are relatively straightforward to diagnose, there may be a risk of overdiagnosis of Type 1 because of an overlap within the normal range. We also report on the clinical profile and diagnosis of VWD in a South American cohort of patients and on the in vitro characteristics of some factor concentrates available for treatment of VWD.
Favaloro
E J
EJ
Institute of Clinical Pathology and Medical Research, Westmead, NSW 2145, Australia. emmanuel@icpmr.wsahs.nsw.gov.au
Lillicrap
D
D
Lazzari
M A
MA
Cattaneo
M
M
Mazurier
C
C
Woods
A
A
Meschengieser
S
S
Blanco
A
A
Kempfer
A C
AC
Hubbard
A
A
Chang
A
A
D000293:Adolescent; D000328:Adult; D001792:Blood Platelets / Q000737:chemistry; D015331:Cohort Studies; D005169:Factor VIII / Q000032:analysis / Q000627:therapeutic use; D005192:Family Health; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000097:blood; D012307:Risk Factors; D014842:von Willebrand Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D014841:von Willebrand Factor / Q000032:analysis
D014841:von Willebrand Factor; D005169:Factor VIII