Lower doses of rFVIIa therapy are safe and effective for surgical interventions in patients with severe FXI deficiency and inhibitors

Kenet, Gili; Lubetsky, Aaron; Luboshitz, Jacob; Ravid, B.; Tamarin, Ilia; Varon, David; Martinowitz, Uri

doi:10.1111/j.1365-2516.2009.02043.x

Cited by 33 publications

(25 citation statements)

References 54 publications

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“…733 Low-dose rFVIIa (33 to 47 mg kg À1 ) also appears to be well tolerated and effective for surgery in patients with severe factor XI deficiency and inhibitors. 734 Coadministration of tranexamic acid has also proved effective, 735,736 although it may increase thrombotic risks. Elsewhere, effective haemostasis was reported in 100% of factor XI deficient patients receiving prophylactic rFVIIa before dental procedures, and minor or major surgery.…”

Section: Rare Bleeding Disorders Recommendationsmentioning

confidence: 99%

Management of severe perioperative bleeding

Kozek‐Langenecker

Ahmed

Afshari

et al. 2017

European Journal of Anaesthesiology

694

316

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: The management of perioperative bleeding involves multiple assessments and strategies to ensure appropriate patient care. Initially, it is important to identify those patients with an increased risk of perioperative bleeding. Next, strategies should be employed to correct preoperative anaemia and to stabilise macrocirculation and microcirculation to optimise the patient's tolerance to bleeding. Finally, targeted interventions should be used to reduce intraoperative and postoperative bleeding, and so prevent subsequent morbidity and mortality. The objective of these updated guidelines is to provide healthcare professionals with an overview of the most recent evidence to help ensure improved clinical management of patients. For this update, electronic databases were searched without language restrictions from 2011 or 2012 (depending on the search) until 2015. These searches produced 18 334 articles. All articles were assessed and the existing 2013 guidelines were revised to take account of new evidence. This update includes revisions to existing recommendations with respect to the wording, or changes in the grade of recommendation, and also the addition of new recommendations. The final draft guideline was posted on the European Society of Anaesthesiology website for four weeks for review. All comments were collated and the guidelines were amended as appropriate. This publication reflects the output of this work.

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Section: Rare Bleeding Disorders Recommendationsmentioning

confidence: 99%

Management of severe perioperative bleeding

Kozek‐Langenecker

Ahmed

Afshari

et al. 2017

European Journal of Anaesthesiology

694

316

View full text Add to dashboard Cite

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“…Reported interventions include immunosuppression (Teruya & Styler, 2000), plasma exchange (Teruya & Styler, 2000). rFVIIa 15-30 lg/kg in combination with tranexamic acid prevented bleeding in major surgery in cases with FXI inhibitors, without venous thrombosis (Kenet et al, 2009;Livnat et al, 2009).…”

Section: Fxi Inhibitorsmentioning

confidence: 99%

Guideline for the diagnosis and management of the rare coagulation disorders

et al. 2014

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“…The use of rFVIIa at low doses (15-30 μg/kg) seems to be a safe treatment regimen in patients with SFXID and inhibitors undergoing surgeries. 65…”

Section: Thrombosis In Factor VII Deficiencymentioning

confidence: 99%

“…61 Occasionally seen in patients treated by standard dose of rFVIIa. 65 Arterial events mainly myocardial infarction. 11 Anecdotal cases of venous thrombosis.…”

Section: Recommendations For Managementmentioning

confidence: 99%

Occurrence of Thrombosis in Rare Bleeding Disorders

Ruiz-Sáez¹

2013

Semin Thromb Hemost

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Paradoxically, there are reports of thrombotic events for some rare bleeding disorders associated with significant bleeding tendency. Afibrinogenemia, factor (F) VII, or FXI deficiencies are those most commonly associated with venous or arterial thrombosis. Pathogenesis is multifactorial and the main conditions associated with this complication relate to the coexistence of inherited or acquired thrombotic risk factors linked to certain specific characteristics of the underlying defect. Patients with afibrinogenemia can develop severe, spontaneous, or recurrent thromboembolic disease. Up to 20% of congenital dysfibrinogenemia patients show predisposition to thrombosis. Thrombotic episodes, particularly deep vein thrombosis, have been reported in 3 to 4% FVII deficient patients, even those who were severely affected. These events have been reported either after infusion of plasma derived FXI concentrate or recombinant activated FVII in FXI deficient patients. So, in addition to factor level, replacement therapy must be individualized and should take into account past personal or family history of bleeding and thrombosis, and other prothrombotic risk factors. Treatment of thrombosis represents a challenge. For mild factor deficiencies, antithrombotic prophylaxis must be considered with or without concomitant use of replacement therapy. For all patients, it is also recommended to control known cardiovascular disease risk factors.

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Lower doses of rFVIIa therapy are safe and effective for surgical interventions in patients with severe FXI deficiency and inhibitors

Cited by 33 publications

References 54 publications

Management of severe perioperative bleeding

Management of severe perioperative bleeding

Guideline for the diagnosis and management of the rare coagulation disorders

Occurrence of Thrombosis in Rare Bleeding Disorders

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