01-10-2021 | Hemophilia | Case Report
Diagnostic Dilemma in Peripartum Management of von Willebrand Disease: a Case Report
Published in: The Journal of Obstetrics and Gynecology of India | Issue 5/2021
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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.
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
|