Desmopressin for the treatment of haemophilia

The synthetic vasopressin analogue (1–deamino‐8‐d‐arginine‐vasopressin) increases plasma concentration of factor VIII and von Willebrand factor in normal subjects and patients with mild haemophilia A and von Willebrand disease. Since its first clinical use in 1977, desmopressin has become the treatm...

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Bibliographic Details
Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2008-01, Vol.14 (s1), p.15-20
Main Author: CASTAMAN, G.
Format: Article
Language:English
Subjects:
Administration, Intranasal
Adult
biologic response
Child
Deamino Arginine Vasopressin - administration & dosage
Deamino Arginine Vasopressin - therapeutic use
factor VIII
haemophilia
Hemophilia A - drug therapy
Hemostatics - administration & dosage
Hemostatics - therapeutic use
Humans
inherited bleeding disorders
Injections, Intravenous
von Willebrand factor
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Summary:The synthetic vasopressin analogue (1–deamino‐8‐d‐arginine‐vasopressin) increases plasma concentration of factor VIII and von Willebrand factor in normal subjects and patients with mild haemophilia A and von Willebrand disease. Since its first clinical use in 1977, desmopressin has become the treatment of choice for patients with haemophilia A and factor VIII coagulant activity (FVIII:C) > 5% and has spared several patients the risk of acquiring blood‐borne viral infections due to the use of non‐virally inactivated plasma‐derived FVIII concentrates. An average two to sixfolds FVIII:C increase is typically observed in most patients and return to baseline occurs usually within 8 hours. Several clinical studies have demonstrated the clinical efficacy and safety of desmopressin and the availability of concentrated formulation for subcutaneous injection and of a nasal spray has paved the way to home‐treatment. However, overall it appears that haemophilic children may have a lower rate of biologic response compared to adults and a minority of adult patients are not able to attain clinically useful FVIII:C levels post‐desmopressin administration. Thus, in every patient with haemophilia A likely to be treated or candidate to an elective invasive procedure, a test‐infusion/injection should be carried out to assess the future usefulness of the compound.
ISSN:1351-8216
1365-2516
DOI:10.1111/j.1365-2516.2007.01606.x