Treatment of the severe bleeding episode in type III von Willebrand??s disease by simultaneous administration of cryoprecipitate and platelet concentrate

Boda, Zoltán; Pfliegler, G; Hársfalvi, Jolán; K, Rák

doi:10.1097/00001721-199112000-00013

Cited by 13 publications

(11 citation statements)

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“…Platelet transfusions may be required in patients experiencing excessive and recurrent bleeding (e.g. gastrointestinal) despite treatment with VWF/FVIII concentrates and have been shown to be effective in some, if not all, patients [65,66,67]. …”

Section: Adjuvant Treatmentsmentioning

confidence: 99%

Current Management of Patients with Severe von Willebrand Disease Type 3: A 2012 Update

Federici

James²

2012

Acta Haematol

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Von Willebrand disease type 3 (VWD3) is the most severe form of this bleeding disorder due to the almost complete deficiency of von Willebrand factor (VWF). VWD3 is inherited as an autosomal recessive trait. While heterozygous carriers exhibit mild or no bleeding symptoms, most patients with VWD3, which is characterized by undetectable levels of VWF antigen (VWF:Ag) and reduced concentrations (<20 IU/dl) of factor VIII (FVIII), show severe bleeding symptoms. Although the incidence of VWD3 is rare, the condition is of considerable interest because of its severe clinical presentation, the need for replacement therapy and the risk of alloantibodies following infusion of plasma-derived VWF concentrates. This review, based on clinical experience, provides an update on the clinical, laboratory and molecular markers of VWD3 that can be useful for determining the optimal therapeutic approach in these patients.

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Section: Adjuvant Treatmentsmentioning

confidence: 99%

Current Management of Patients with Severe von Willebrand Disease Type 3: A 2012 Update

Federici

James²

2012

Acta Haematol

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“…When hemorrhage persists despite high VWF/FVIII levels, administration of platelet concentrates can be helpful, emphasizing the important role of platelet VWF in maintaining primary hemostasis. [59][60][61] Platelet concentrates are effective, particularly in patients with type 3 VWD, probably because of their role in transporting VWF to sites of vascular injury where they secrete actively prothrombotic VWF.…”

Section: Desmopressin (1-deamino-8-d-arginine Vasopressin [Ddavp]mentioning

confidence: 99%

von Willebrand factor to the rescue

Meyer

Deckmyn

Vanhoorelbeke

2009

Blood

126

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“…When the bleeding episode cannot be managed despite high FVIII levels, platelet concentrates are given together with plasma products to control bleeding [43][44][45]. Platelet concentrates are effective, particularly in patients with type 3 VWD, most likely because these platelets transport VWF to sites of vascular injury, after which they secrete VWF locally where needed.…”

Section: Vwf Concentratesmentioning

confidence: 99%

Von Willebrand Factor: Drug and Drug Target

Meyer¹,

Maeyer²,

Deckmyn³

et al. 2009

CHDDT

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One of the key players in many thrombotic complications is von Willebrand factor (VWF), a large, multimeric glycoprotein that is present in plasma where it fulfils a crucial role in haemostasis. First, VWF recruits platelets to vascular lesions by acting as a linker molecule between the exposed collagen and free-flowing platelets in the circulation. Second, by serving as a carrier protein for the coagulation factor VIII, VWF protects this anti-haemophilic factor from rapid degradation. Quantitative or qualitative defects in VWF result in the most common bleeding disorder in man, known as von Willebrand disease, illustrating the central role of VWF in haemostasis. On the other hand, a thrombotic risk emerges when over-reactive VWF molecules can bind spontaneously to platelets. It is clear that because of its pivotal role in maintaining the fine balance between bleeding and thrombosis, VWF is an attractive but delicate drug target. This review focuses on the role of VWF in both haemostasis and thrombosis with special attention to the molecule as drug and drug target respectively.

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Treatment of the severe bleeding episode in type III von Willebrand??s disease by simultaneous administration of cryoprecipitate and platelet concentrate

Cited by 13 publications

References 0 publications

Current Management of Patients with Severe von Willebrand Disease Type 3: A 2012 Update

Current Management of Patients with Severe von Willebrand Disease Type 3: A 2012 Update

von Willebrand factor to the rescue

Von Willebrand Factor: Drug and Drug Target

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