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.
Institute of Clinical Pathology and Medical Research, Westmead, NSW 2145, Australia. emmanuel@icpmr.wsahs.nsw.gov.au
E J
EJ
Favaloro
D
D
Lillicrap
M A
MA
Lazzari
M
M
Cattaneo
C
C
Mazurier
A
A
Woods
S
S
Meschengieser
A
A
Blanco
A C
AC
Kempfer
A
A
Hubbard
A
A
Chang
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