Classification
Three types of von Willebrand's disease are recognised on the basis of ristocetin activity and other tests: (1)
- type I, the the classic type described by von Willebrand in 1926, where the von Willebrand factor is functionally normal but present at low levels (2)
- type II, a broad spectrum of diseases in which bleeding time is prolonged in almost all cases. vWF levels may increase under some conditions - like stress or pregnancy, correcting the bleeding time
- type IIa - both plasma and platelet samples lack the intermediate and high molecular weight vWF multimers; platelets do not aggregate with ristocetin
- type IIb - plasma lacks the highest molecular weight vWF multimers; platelets aggregate in response to low dose ristocetin
- type III is inherited as an autosomal recessive trait. Homozygous individuals have severe bleeding problems related to marked reduction of all von Willebrand's factor parameters and of Factor VIII
Reference
- Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor. J Thromb Haemost. 2006 Oct;4(10):2103-14.
- Peake I, Goodeve A. Type 1 von Willebrand disease. J Thromb Haemost. 2007 Jul;5 Suppl 1:7-11.
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