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Von Willebrand disease - Type 2B

Belurkar, Sushma V and Manohar, Chethan and Kurien, Annamma (2013) Von Willebrand disease - Type 2B. Journal of Evolution of Medical and Dental Sciences, 2 (3). pp. 240-244.

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Abstract

Type 2B Von Willebrand disease (vWD) is a rare type of vWD accounting for less than 20 % of the cases of vWD. The characteristic feature of this condition is increased affinity of vWF for the glycoprotein Ib-IX-V complex on platelets resulting in clearing of the high molecular weight vWF multimers and platelets from the circulation. So the patient will present with low levels of vWF as well as thrombocytopenia. This functional abnormality can be demonstrated by hyperaggregation seen with low dose ristocetin on platelet aggregation. Here we present a case of type 2B vWD in a seven year old male child who presented with skin bleeds and prolonged bleeding following tooth fall. The case is discussed because of its rarity and to emphasize the careful laboratory evaluation required to avoid misdiagnosis in such cases

Item Type: Article
Uncontrolled Keywords: Von Willebrand factor; GlycoproteinIb, Ristocetin
Subjects: Medicine > MMMC Manipal > Pathology
Medicine > KMC Manipal > Pathology
Depositing User: KMC Manipal
Date Deposited: 25 Mar 2013 06:21
Last Modified: 16 Sep 2015 12:38
URI: http://eprints.manipal.edu/id/eprint/79270

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