Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors

Ampaiwan Chuansumrit1, Pantep Angchaisuksiri2, Nongnuch Sirachainan11Departments of Pediatrics and 2Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University,  Bangkok, ThailandAbstract: Hemophilia patients with inhibitors faced the constraint of inadequate treatment for s...

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Main Authors: Ampaiwan Chuansumrit, Pantep Angchaisuksiri, Nongnuch Sirachainan
Format: Article
Language:English
Published: Dove Medical Press 2010-03-01
Series:Journal of Blood Medicine
Online Access:http://www.dovepress.com/critical-appraisal-of-the-role-of-recombinant-activated-factor-vii-in--a4159
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author Ampaiwan Chuansumrit
Pantep Angchaisuksiri
Nongnuch Sirachainan
author_facet Ampaiwan Chuansumrit
Pantep Angchaisuksiri
Nongnuch Sirachainan
author_sort Ampaiwan Chuansumrit
collection DOAJ
description Ampaiwan Chuansumrit1, Pantep Angchaisuksiri2, Nongnuch Sirachainan11Departments of Pediatrics and 2Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University,  Bangkok, ThailandAbstract: Hemophilia patients with inhibitors faced the constraint of inadequate treatment for several years before the era of recombinant factor VIIa (rFVII). Initially, rFVIIa was used in the compassionate-use programs. After a worldwide license was issued, more than 1.5 million doses were administered. Bleeding of joints and muscles was controlled effectively by means of an early home treatment program, with either a standard dose of 90 μg/kg every 2 to 3 hours for a few doses or a single dose of 270 μg/kg. For more serious bleeding episodes or minor surgery, an initial dose of 90 μg/kg was given every 2 hours for 24 to 48 hours followed by increased intervals of 3 to 6 hours according to the severity of bleeding and efficacy of bleeding control. In cases of major surgery such as orthopedic procedures, the same regimen can be applied except for a higher initial dose of 120 to 180 μg/kg. However, increasing the dose should be considered if there are unexpected bleeding complications since the half-life and clearance of rFVIIa differ between individuals. In addition, prophylaxis is administered to a small number of patients. Finally, the reported thromboembolic events found in hemophilia patients with inhibitors receiving rFVIIa are extremely low, much less than 1%.Keywords: bleeding disorder, hemophilia, inhibitor, NovoSeven, recombinant factor VIIa
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spelling doaj.art-275fd560459148858c2d927885c1fd9b2022-12-21T22:10:59ZengDove Medical PressJournal of Blood Medicine1179-27362010-03-012010default3748Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitorsAmpaiwan ChuansumritPantep AngchaisuksiriNongnuch SirachainanAmpaiwan Chuansumrit1, Pantep Angchaisuksiri2, Nongnuch Sirachainan11Departments of Pediatrics and 2Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University,  Bangkok, ThailandAbstract: Hemophilia patients with inhibitors faced the constraint of inadequate treatment for several years before the era of recombinant factor VIIa (rFVII). Initially, rFVIIa was used in the compassionate-use programs. After a worldwide license was issued, more than 1.5 million doses were administered. Bleeding of joints and muscles was controlled effectively by means of an early home treatment program, with either a standard dose of 90 μg/kg every 2 to 3 hours for a few doses or a single dose of 270 μg/kg. For more serious bleeding episodes or minor surgery, an initial dose of 90 μg/kg was given every 2 hours for 24 to 48 hours followed by increased intervals of 3 to 6 hours according to the severity of bleeding and efficacy of bleeding control. In cases of major surgery such as orthopedic procedures, the same regimen can be applied except for a higher initial dose of 120 to 180 μg/kg. However, increasing the dose should be considered if there are unexpected bleeding complications since the half-life and clearance of rFVIIa differ between individuals. In addition, prophylaxis is administered to a small number of patients. Finally, the reported thromboembolic events found in hemophilia patients with inhibitors receiving rFVIIa are extremely low, much less than 1%.Keywords: bleeding disorder, hemophilia, inhibitor, NovoSeven, recombinant factor VIIahttp://www.dovepress.com/critical-appraisal-of-the-role-of-recombinant-activated-factor-vii-in--a4159
spellingShingle Ampaiwan Chuansumrit
Pantep Angchaisuksiri
Nongnuch Sirachainan
Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors
Journal of Blood Medicine
title Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors
title_full Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors
title_fullStr Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors
title_full_unstemmed Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors
title_short Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors
title_sort critical appraisal of the role of recombinant activated factor vii in the treatment of hemophilia patients with inhibitors
url http://www.dovepress.com/critical-appraisal-of-the-role-of-recombinant-activated-factor-vii-in--a4159
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AT nongnuchsirachainan criticalappraisaloftheroleofrecombinantactivatedfactorviiinthetreatmentofhemophiliapatientswithinhibitors