Question 8.2 from the first paper of 2013 presents the candidates with a coagulopathy scenario where the patient has a normal APTT but an abnormally prolonged bleeding time. Because Factor 8 relies on vWF activity, and in the absence of vWF one is effectively Factor 8-deficient, the APTT may be slightly prolonged. The PT would be normal. An excellent 2013 article by Castaman et al has been pillaged for the details that make up this summary.
Pathophysiology
There are three main types of VWD:
History and examination
Blood biochemistry
VWF level and FVIII level are the tests which correlate with severity of disease; the other two tests discriminate between different subtypes of Type 2 VWD.
Management
Budde, Ulrich, et al. "Laboratory diagnosis of congenital von Willebrand disease."Seminars in thrombosis and hemostasis. Vol. 28. No. 02. Copyright© 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.:+ 1 (212) 584-4662, 2002.
Kamal, Arif H., Ayalew Tefferi, and Rajiv K. Pruthi. "How to interpret and pursue an abnormal prothrombin time, activated partial thromboplastin time, and bleeding time in adults." Mayo Clinic Proceedings. Vol. 82. No. 7. Elsevier, 2007.
Castaman, Giancarlo, Anne Goodeve, and Jeroen Eikenboom. "Principles of care for the diagnosis and treatment of von Willebrand disease." Haematologica 98.5 (2013): 667-674.