NovoSeven® in immune tolerance therapy

Brackmann, H.‐H.; Effenberger, E.; Hess, L.; Schwaab, R.; Oldenburg, Johannes

doi:10.1097/00001721-200004001-00009

Cited by 42 publications

(47 citation statements)

References 11 publications

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“…Since rfVIIa is a recombinant product and has no potential for anamnesis to fVIII (small amounts of fVIII can be found in FEIBA VH), 44 we favor the initial use of rfVIIa as a bypassing agent for acute management of bleeding episodes in patients with inhibitors that no longer respond to fVIII. For typical joint bleeds we begin treatment with standard doses, 90 to 120 mcg/kg rounded up to the nearest vial size, given every 2 to 3 hours.…”

Section: Treatment Of Acute Bleedingmentioning

confidence: 99%

“…In patients with a newly diagnosed inhibitor in which the inhibitor titer is greater than 10 BU/mL before starting ITI, we use bypassing agents (preferably rfVIIa) until the inhibitor is less than 10 BU/mL. 44 Ideally, once the inhibitor titer is less than 10 BU/mL, ITI is initiated without delay. However, because of the high degree of commitment required, not all patients and families are suitable to begin ITI at the time a nontransient inhibitor is diagnosed.…”

Section: Long-term Eradication Of Inhibitory Antibodiesmentioning

confidence: 99%

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How we treat a hemophilia A patient with a factor VIII inhibitor

Kempton

White

2009

Blood

150

144

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The most significant complication of treatment in patients with hemophilia A is the development of alloantibodies that inhibit factor VIII activity. In the presence of inhibitory antibodies, replacement of the missing clotting factor by infusion of factor VIII becomes less effective. Once replacement therapy is ineffective, acute management of bleeding requires agents that bypass factor VIII activity. Long-term management consists of eradicating the inhibitor through immune tolerance. Despite success in the treatment of acute bleeding and inhibitor eradication, there remains an inability to predict or prevent inhibitor formation. Ideally, prediction and ultimately prevention will come with an improved understanding of how patientspecific and treatment-related factors work together to influence anti-factor VIII antibody production. (Blood. 2009;113: 11-17)

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Section: Treatment Of Acute Bleedingmentioning

confidence: 99%

Section: Long-term Eradication Of Inhibitory Antibodiesmentioning

confidence: 99%

How we treat a hemophilia A patient with a factor VIII inhibitor

Kempton

White

2009

Blood

150

144

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“…Bypassing agents have also been employed for secondary prophylaxis in patients with frequently recurrent bleeding episodes, but risk-efficacy and cost-benefit evaluations are still missing as well as the optimal dose and administration schedule [101][102][103][104].…”

Section: Treatment Of Bleeding and Prophylaxismentioning

confidence: 99%

Italian guidelines for the diagnosis and treatment of patients with haemophilia and inhibitors

Gringeri

Mannucci

2005

Haemophilia

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The Italian Association of Haemophilia Centres reviewed and finally approved in November 2004 the new Italian Guidelines for the diagnosis and treatment of patients with clotting factor inhibitors. The recommendations have been based on the identification of levels of clinical evidence derived from the systematic review carried out in 2003 by the School of Health and Related Research, the University of Sheffield, UK, and further integrated by clinical studies published from 2003 to 2004. The Italian guidelines consist of six major domains concerning inhibitor definition, epidemiology, risk factors, diagnosis, inhibitor eradication, management of bleeding episodes, in patients with congenital and acquired coagulation disorders, with 121 statements, 59 synthesis and 54 recommendations. We report here recommendations and open issues concerning the diagnosis and monitoring of inhibitors, inhibitor eradication and the management of bleeding in patients with haemophilia A and B.

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“…Immune tolerance protocols have been developed that are successful in approximately 80% of children. [72][73][74][75] Higher success rates are seen when the inhibitor titer is allowed to decline to a low level, preferably < 10 BU, before commencing immune tolerance therapy. In addition, central venous access is necessary to facilitate the administation by parents of frequent high-dose factor concentrate.…”

Section: Inhibitors and Their Treatmentmentioning

confidence: 99%