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14 - Inherited disorders of primary hemostasis

from Section 5 - Hemorrhagic disorders

Published online by Cambridge University Press:  06 December 2010

Sue Pavord
Affiliation:
Leicester Royal Infirmary
Beverley Hunt
Affiliation:
Guy's and St Thomas' NHS Foundation Trust
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Summary

The management of inherited bleeding disorders during pregnancy, delivery, and the postpartum period is particularly challenging. This chapter describes the clinical features, disease prevalence, classification, and laboratory evaluation of Von Willebrand disease (VWD). Pregnancy, delivery, and the postpartum period pose significant challenges to the hemostatic system, and women with VWD need to be carefully managed during these at-risk times. Women with VWD have an increased risk of bleeding events and even death during childbirth. DDAVP (1-deamino-8-D-arginine vasopressin) a synthetic derivative of antidiuretic hormone that acts specifically through type 2 vasopressin receptors, stimulates release of ultralarge VWF multimers from storage in the Wiebel Palade bodies of the endothelial cells. Tranexamic acid is a useful adjunct to desmopressin, particularly as it counteracts the mild fibrinolytic effect of DDAVP related to the associated rise in tissue plasminogen activator. Glanzmann's thrombasthenia is a congenitally acquired platelet disorder with an autosomal recessive mode of inheritance.
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Publisher: Cambridge University Press
Print publication year: 2010

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