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Published in: The Journal of Obstetrics and Gynecology of India 5/2021

01-10-2021 | Hemophilia | Case Report

Diagnostic Dilemma in Peripartum Management of von Willebrand Disease: a Case Report

Authors: Megha Garg, Uma Pandey, Lalit Prashant Meena

Published in: The Journal of Obstetrics and Gynecology of India | Issue 5/2021

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Excerpt

Von Willebrand's disease (vWD) is the most common inherited bleeding disorder in humans with an incidence of 1–2% in the general population [1]. Von Willebrand’s disease is an autosomal hereditary bleeding disorder associated with a quantitative or qualitative defect of Von Willebrand factor (vWF) [2]. The vWF transports and stabilises factor VIII and facilitates adhesion of platelets to the endothelium. The disease is classified as type-1 in patients with decreased amount of vWF (quantitative deficiency), type-2 in patients with structural defect of vWF (qualitative deficiency) and type-3 in cases with no vWF function or protein [3]. (Table 1). The incidence of haemophilia A is 1 out of 10,000 male births, while of haemophilia B is 1 out of 30,000 male births with the prevalence ranging from 5.4 to 14.5 per 100,000 male births. The severity of the bleeding symptoms depends on the degree of vWF and FVIII reduction and other factors [4]. The management of vWF disease patients depends on the type of vWF disease, factor levels, symptoms, surgery in future, etc.
Table 1
Classification of von Willebrand disease (VWF)
S.No
Type of von Willebrand disease
Description
1
Type 1
Partial quantitative deficiency of VWF
2
Type 3
Virtually complete deficiency of VWF
3
Type 2
Qualitative deficiency of VWF
 
Type 2A
Qualitative variants with decreased platelet‐dependent function associated with the absence of high and intermediate‐molecular weight VWF multimers
 
Type 2B
Qualitative variants with increased affinity for platelet GPIb
 
Type 2M
Qualitative variants with decreased platelet‐dependent function not caused by the absence of high‐molecular weight VWF multimers
Literature
1.
go back to reference Abu-Douleh E, Al-Numair N, Albanyan A, Alsuliman A, Bayoumi N, Owaidah T. Prevalence of von Willebrand disease among university students in Riyadh, Saudi Arabia. J Appl Hematol 2018;9:136–9. Abu-Douleh E, Al-Numair N, Albanyan A, Alsuliman A, Bayoumi N, Owaidah T. Prevalence of von Willebrand disease among university students in Riyadh, Saudi Arabia. J Appl Hematol 2018;9:136–9.
2.
go back to reference Michiels JJ, Batorova A, Prigancova T, Smejkal P, Penka M, Vangenechten I, Gadisseur A. Changing insights in the diagnosis and classification of autosomal recessive and dominant von Willebrand diseases 1980–2015. World J Hematol 2016;5(3):61–74. Michiels JJ, Batorova A, Prigancova T, Smejkal P, Penka M, Vangenechten I, Gadisseur A. Changing insights in the diagnosis and classification of autosomal recessive and dominant von Willebrand diseases 1980–2015. World J Hematol 2016;5(3):61–74.
3.
go back to reference Bhatt RK, et al. Pregnancy and delivery of a women with Von Willebrand disease type 3: a case report. Int J Reprod Contracept Obstet Gynecol. 2020;9(2):881–3.CrossRef Bhatt RK, et al. Pregnancy and delivery of a women with Von Willebrand disease type 3: a case report. Int J Reprod Contracept Obstet Gynecol. 2020;9(2):881–3.CrossRef
4.
go back to reference Lillicrap D, Dean J, Blanchette VS. von Willebrand disease. In: Lilleyman J, Hann I, Blanchette V, editors. Pediatric Hematology. Philadelphia: Churchill Livingstone; 2000. p. 601–9. Lillicrap D, Dean J, Blanchette VS. von Willebrand disease. In: Lilleyman J, Hann I, Blanchette V, editors. Pediatric Hematology. Philadelphia: Churchill Livingstone; 2000. p. 601–9.
Metadata
Title
Diagnostic Dilemma in Peripartum Management of von Willebrand Disease: a Case Report
Authors
Megha Garg
Uma Pandey
Lalit Prashant Meena
Publication date
01-10-2021
Publisher
Springer India
Published in
The Journal of Obstetrics and Gynecology of India / Issue 5/2021
Print ISSN: 0971-9202
Electronic ISSN: 0975-6434
DOI
https://doi.org/10.1007/s13224-021-01438-5

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