Hyper‐responsiveness to DDAVP for patients with type I von Willebrand's disease and normal intra‐platelet von Willebrand factor

Rodeghiero, Francesco; Castaman, Giancarlo; Bona, Eros Di; Ruggeri, Marco; Lombardi, Rossana; Pm, Mannucci

doi:10.1111/j.1600-0609.1988.tb00815.x

Cited by 38 publications

(10 citation statements)

References 19 publications

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“…Grade B, level IIb [141,143,145,146,148,154,155] • Topical agents, such as fibrin sealant or bovine thrombin, may be useful adjuncts for oral surgery in persons who have VWD.…”

Section: Expert Commentarymentioning

confidence: 99%

An update on type 2B von Willebrand disease

Mikhail

Aldin

Streiff

et al. 2014

Expert Review of Hematology

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Type 2B von Willebrand disease (VWD) accounts for fewer than 5% of all VWD patients. In this disease, mutations in the A1 domain result in increased von Willebrand factor (VWF) binding to platelet GPIbα receptors, causing increased platelet clearance and preferential loss of high molecular weight VWF multimers. Diagnosis is complicated because of significant clinical variations even among patients with identical mutations. Platelet transfusion often provides suboptimal results since transfused platelets may be aggregated by the patients' abnormal VWF. Desmopressin may cause a transient decrease in platelet count that could lead to an increased risk of bleeding. Replacement therapy with factor VIII/VWF concentrates is the most effective approach to prevention and treatment of bleeding in type 2B VWD.

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“…Grade B, level IIb [141,143,145,146,148,154,155] • Topical agents, such as fibrin sealant or bovine thrombin, may be useful adjuncts for oral surgery in persons who have VWD.…”

Section: Expert Commentarymentioning

confidence: 99%

An update on type 2B von Willebrand disease

Mikhail

Aldin

Streiff

et al. 2014

Expert Review of Hematology

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“…Table 12 displays published reports of desmopressin effects on laboratory assays of VWF and FVIII in normal persons and persons who have various subtypes of VWD [66,94,101,218–229]. When administered intravenously (i.v.)…”

Section: Management Of Vwdmentioning

confidence: 99%

von Willebrand disease (VWD): evidence‐based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA)¹

et al. 2008

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Summary. von Willebrand disease (VWD) is a commonly encountered inherited bleeding disorder affecting both males and females, causing mucous membrane and skin bleeding symptoms, and bleeding with surgical or other haemostatic challenges. VWD may be disproportionately symptomatic in women of child‐bearing age. It may also occur less frequently as an acquired disorder (acquired von Willebrand syndrome). VWD is caused by deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates platelet haemostatic function and stabilizes blood coagulation factor VIII. The pathophysiology, classification, diagnosis and management of VWD are relatively complex, but understanding them is important for proper diagnosis and management of patients with VWD. These evidence‐based guidelines for diagnosis and management of VWD from the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel (USA) review relevant publications, summarize current understanding of VWD pathophysiology and classification, and present consensus diagnostic and management recommendations based on analysis of the literature and expert opinion. They also suggest an approach for clinical and laboratory evaluation of individuals with bleeding symptoms, history of bleeding or conditions associated with increased bleeding risk. This document summarizes needs for further research in VWF, VWD and bleeding disorders, including clinical research to obtain more objective information about bleeding symptoms, advancements in diagnostic and therapeutic tools, and enhancement in the education and training of clinicians and scientists in bleeding and thrombotic disorders. The NHLBI Web site (http://www.nhlbi.nih.gov/guidelines/vwd) has a more detailed document, a synopsis of these recommendations, and patient education information.

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“…43 Desmopressin administration is the treatment of choice for VWD type 1 because functionally intact VWF is present in endothelial storage granules of these patients. 44,45 However, it is not effective in VWD type 3 or in severe cases of VWD type 1, yields a variable response in types 2A and 2M, and is contraindicated in patients with VWD type 2B because of the possibility of inducing transient thrombocytopenia. In view of the range in responses to DDAVP, a test infusion is recommended in all VWD patients (except type 2B).…”

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

confidence: 99%