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Desmopressin acetate as a haemostatic elevator in individuals with combined deficiency of factors V and VIII : a clinical trial
Author(s) -
Mansouritorghabeh H.,
Shirdel A.
Publication year - 2016
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/jth.13207
Subject(s) - desmopressin , medicine , coagulation , fresh frozen plasma , endocrinology , gastroenterology , platelet
Essentials Combined factor V (FV) and factor VIII (FVIII) deficiency (CF5F8D) is an autosomal recessive coagulation disorder. Desmopressin acetate (DDAVP) was intravenously infused in 20 adult patients with CF5F8D. DDAVP can enhance FVIII levels but has no effect on FV levels in patients with CF5F8D. DDAVP can be substituted for FVIII concentrates in patients with CF5F8D.Summary Objectives Combined factor V (FV) and FVIII deficiency ( CF 5F8D) is a rare inherited autosomal recessive double‐gene disorder most frequently seen in the Middle East. Although affected individuals have deficiency of two coagulation factors (range 5–30%), their bleeding tendencies are similar to patients who have deficiency of a single coagulation factor at the same level. The mainstay of their treatment is infusion of FVIII concentrate and fresh frozen plasma. Here, the effect of intravenous infusion of desmopressin acetate ( DDAVP ) on elevation of coagulation FV and FVIII was investigated through a clinical trial in May 2015. Patients and methods In a registered controlled trial, DDAVP (dosage 0.3 μg kg −1 ) was intravenously infused into 20 adult patients with CF 5F8D over 20 min. After an hour, blood samples were collected and plasma levels of FV and FVIII were measured. Results and conclusion This study revealed that DDAVP can enhance FVIII levels but has no effect on FV plasma concentration in patients with CF 5F8D. Based on these findings, FVIII concentrates may be substituted for DDAVP in patients with CF 5F8D.