Desmopressin in treatment of haematological disorders and in prevention of surgical bleeding

Svensson, Peter; Bergqvist, Peter B. F.; Juul, Kristian Vinter; Berntorp, Erik

doi:10.1016/j.blre.2014.03.001

Cited by 65 publications

(51 citation statements)

References 61 publications

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“…Even if the patient’s bleeding symptoms are reduced by treatment, DDAVP is recognized to reduce bleeding symptoms in subjects without VWD who might have platelet function defects, uremia, or liver disease. 6 Thus, establishing a cause and effect relationship between VWF and bleeding is not necessarily simple. This observation has been repeatedly emphasized by Evan Sadler.…”

Section: Zpmcb-vwdmentioning

confidence: 99%

What have we learned from large population studies of von Willebrand disease?

Montgomery

Flood

2016

Hematology

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Von Willebrand factor (VWF) is a critical regulator of hemostatic processes, including collagen binding, platelet adhesion, and platelet aggregation. It also serves as a carrier protein to normalize plasma factor VIII synthesis, release, and survival. While VWF protein measurements by immunoassay are reasonably comparable between institutions, the measurement of VWF ristocetin cofactor activity (VWF:RCo) has significant variability. Other tests of VWF function, including collagen binding or platelet glycoprotein IIb–IIIa binding, are not universally available, yet these functional defects may cause major bleeding even with normal VWF antigen (VWF:Ag) and VWF:RCo assays. This results in both the overdiagnosis and underdiagnosis of VWD. Newer assays of VWF function (using recombinant glycoprotein Ib rather than whole platelets) have been developed that may improve interlaboratory variability. Some of these tests are not uniformly available and may not be licensed in the United States. Large longitudinal studies of VWF in von Willebrand disease (VWD) patients are not available. Patients are sometimes diagnosed with a single diagnostic VWF panel. Plasma VWF levels increase with age, but it is not clear if this results in less bleeding or whether different normal ranges should be used to identify age-related decreases in VWF. In order to quantitatively compare bleeding symptoms in VWD patients and normal individuals, recent studies in the European Union, Canada, United Kingdom, Holland, and the United States have used semiquantitative bleeding assessment tools (BATs). Even with careful centralized testing, including functional assays of VWF, addition of a BAT does not solve all of the problems with VWD diagnosis. No matter where the line is drawn for diagnosis of VWD, VWF is still a continuous variable. Thus, VWD can be a severe hemorrhagic disease requiring frequent treatment or a mild condition that may not be clinically relevant. As will be discussed by Dr. Goodeve in her presentation, genetics has helped us to diagnose type 2 functional variants of VWD but has not been helpful for the many patients who are at the interface of normal and low VWF and carry the possible diagnosis of type 1 VWD. The he-matologist’s management of patients with reduced levels of VWF still requires both the art and science of clinical medicine.

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Section: Zpmcb-vwdmentioning

confidence: 99%

What have we learned from large population studies of von Willebrand disease?

Montgomery

Flood

2016

Hematology

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“…23 The use of desmopressin (DDAVP) has also been suggested for mild dysfunction of MA. 24 This is because the use of DDAVP increases the release of factor VIII, acting much higher on the coagulation cascade, leading ultimately to platelet activation.…”

Section: Thromboelastography Interpretation For Diagnosis and Managementmentioning

confidence: 99%

Thromboelastography: A Practice Summary for Nurse Practitioners Treating Hemorrhage

Bose

Hravnak

2015

The Journal for Nurse Practitioners

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Nurse practitioners may manage patients with coagulopathic bleeding which can lead to life-threatening hemorrhage. Routine plasma-based tests such as prothrombin time and activated partial thromboplastin time are inadequate in diagnosing hemorrhagic coagulopathy. Indiscriminate administration of fresh frozen plasma, platelets or cryoprecipitate for coagulopathic states can be extremely dangerous. The qualitative analysis that thromboelastography provides can facilitate the administration of the right blood product, at the right time, thereby permitting the application of goal-directed therapy for coagulopathic intervention application and patient survival.

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“…Common management of VWD depends upon increasing the circulating concentration of functional VWF, either endogenous or exogenous. Desmopressin acetate (DDAVP) increases endogenous levels but can have important side effects, some of which are dependent upon the dosing regimen [Svensson et al 2014]. Is there an optimal DDAVP dose for each patient in each situation?…”

Section: Introductionmentioning

confidence: 99%

Current controversies in the diagnosis and management of von Willebrand disease

Neff

2015

Therapeutic Advances in Hematology

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Von Willebrand disease (VWD) is the most common inherited bleeding disorder in the world. The spectrum of VWD spans quantitative and qualitative deficiencies of von Willebrand factor (VWF), a platelet adhesive protein. It manifests primarily as mucocutaneous bleeding, but severely affected patients may suffer soft tissue bleeding and hemarthroses. There is disagreement in the multiple guidelines published regarding diagnosis, especially of type 1 VWD, which also remains the most opaque with respect to molecular characterization. Treatment with desmopressin (DDAVP) is most effective in type 1 VWD, but regimens are not standardized. It is not clear which type 2 VWD patients with qualitative deficiencies can be treated with DDAVP and which ones should receive VWF concentrates. No guidelines stipulate which patients might benefit from prophylactic VWF infusions and how they should be dosed. These are some current controversies in VWD that are discussed in this review.

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Desmopressin in treatment of haematological disorders and in prevention of surgical bleeding

Cited by 65 publications

References 61 publications

What have we learned from large population studies of von Willebrand disease?

What have we learned from large population studies of von Willebrand disease?

Thromboelastography: A Practice Summary for Nurse Practitioners Treating Hemorrhage

Current controversies in the diagnosis and management of von Willebrand disease

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