Tags

Type your tag names separated by a space and hit enter

The use of desmopressin in von Willebrand disease: the experience of the first 30 years (1977-2007).
Haemophilia 2008; 14 Suppl 1:5-14H

Abstract

The aim of the treatment for von Willebrand disease (VWD) is to correct the dual defect of haemostasis, i.e. the abnormal platelet adhesion as a result of reduced and/or dysfunctional von Willebrand factor (VWF) and the abnormal coagulation expressed by low levels of factor VIII (FVIII). Correction of both deficiencies can be achieved by administering the synthetic peptide desmopressin (DDAVP) or, in cases unresponsive to this agent, the plasma concentrates containing VWF and FVIII (VWF/FVIII). DDAVP is the treatment of choice for type 1 VWD because it can induce release of normal VWF from cellular compartments, but the drug can be clinically useful also in other VWD types, including acquired von Willebrand syndrome (AVWS). A test dose of DDAVP at the time of diagnosis is recommended to establish the individual patterns of biological response and to predict clinical efficacy during bleeding and surgery. DDAVP is not effective in VWD type 3 and in severe forms of VWD 1 and 2. It can induce transient thrombocytopenia in patients with VWD type 2B. The results of several retrospective studies on the use of DDAVP in VWD management have been reported by many authors in different countries for the last 30 years. However, despite the widespread use of DDAVP in the treatment of VWD, there are only a few prospective clinical trials in a large number of cases on DDAVP efficacy and safety aimed at determining benefits and limits of this therapeutic approach. An investigator-driven observational prospective study on clinical efficacy of DDAVP in 200 patients with VWD types 1 and 2 has been recently organized: the effectiveness and safety of DDAVP will be evaluated prospectively for 24 months during bleeding episodes and minor or major surgeries in the VWD patients who were exposed to an infusion trial at enrollment.

Authors+Show Affiliations

Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Department of Medicine and Medical Specialities, IRCCS Maggiore Policlinico Hospital, University of Milan, Milan, Italy. augusto.federici@unimi.it

Pub Type(s)

Historical Article
Journal Article
Review

Language

eng

PubMed ID

18173689

Citation

Federici, A B.. "The Use of Desmopressin in Von Willebrand Disease: the Experience of the First 30 Years (1977-2007)." Haemophilia : the Official Journal of the World Federation of Hemophilia, vol. 14 Suppl 1, 2008, pp. 5-14.
Federici AB. The use of desmopressin in von Willebrand disease: the experience of the first 30 years (1977-2007). Haemophilia. 2008;14 Suppl 1:5-14.
Federici, A. B. (2008). The use of desmopressin in von Willebrand disease: the experience of the first 30 years (1977-2007). Haemophilia : the Official Journal of the World Federation of Hemophilia, 14 Suppl 1, pp. 5-14. doi:10.1111/j.1365-2516.2007.01610.x.
Federici AB. The Use of Desmopressin in Von Willebrand Disease: the Experience of the First 30 Years (1977-2007). Haemophilia. 2008;14 Suppl 1:5-14. PubMed PMID: 18173689.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The use of desmopressin in von Willebrand disease: the experience of the first 30 years (1977-2007). A1 - Federici,A B, PY - 2008/1/5/pubmed PY - 2008/6/27/medline PY - 2008/1/5/entrez SP - 5 EP - 14 JF - Haemophilia : the official journal of the World Federation of Hemophilia JO - Haemophilia VL - 14 Suppl 1 N2 - The aim of the treatment for von Willebrand disease (VWD) is to correct the dual defect of haemostasis, i.e. the abnormal platelet adhesion as a result of reduced and/or dysfunctional von Willebrand factor (VWF) and the abnormal coagulation expressed by low levels of factor VIII (FVIII). Correction of both deficiencies can be achieved by administering the synthetic peptide desmopressin (DDAVP) or, in cases unresponsive to this agent, the plasma concentrates containing VWF and FVIII (VWF/FVIII). DDAVP is the treatment of choice for type 1 VWD because it can induce release of normal VWF from cellular compartments, but the drug can be clinically useful also in other VWD types, including acquired von Willebrand syndrome (AVWS). A test dose of DDAVP at the time of diagnosis is recommended to establish the individual patterns of biological response and to predict clinical efficacy during bleeding and surgery. DDAVP is not effective in VWD type 3 and in severe forms of VWD 1 and 2. It can induce transient thrombocytopenia in patients with VWD type 2B. The results of several retrospective studies on the use of DDAVP in VWD management have been reported by many authors in different countries for the last 30 years. However, despite the widespread use of DDAVP in the treatment of VWD, there are only a few prospective clinical trials in a large number of cases on DDAVP efficacy and safety aimed at determining benefits and limits of this therapeutic approach. An investigator-driven observational prospective study on clinical efficacy of DDAVP in 200 patients with VWD types 1 and 2 has been recently organized: the effectiveness and safety of DDAVP will be evaluated prospectively for 24 months during bleeding episodes and minor or major surgeries in the VWD patients who were exposed to an infusion trial at enrollment. SN - 1365-2516 UR - https://www.unboundmedicine.com/medline/citation/18173689/The_use_of_desmopressin_in_von_Willebrand_disease:_the_experience_of_the_first_30_years__1977_2007__ L2 - https://doi.org/10.1111/j.1365-2516.2007.01610.x DB - PRIME DP - Unbound Medicine ER -