The use of activated recombinant coagulation factor VII during haemarthroses and synovectomy in a patient with congenital severe factor V deficiency

González-Boullosa, Rosario; Ocampo‐Martínez, R.; Alarcón‐Martín, M. J.; Suárez‐Rodríguez, M.; Domínguez‐Viguera, L.; González‐Fajo, G.

doi:10.1111/j.1365-2516.2005.00956.x

Cited by 27 publications

(25 citation statements)

References 4 publications

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“…Recombinant‐activated factor VII is licensed for the management of bleeding in patients with severe haemophilia and inhibitors and in treating bleeding in patients with severe platelet defects. There are reports of the off label use of rFVIIa in FV deficient patients although none in critical procedures as reported here. The mode of action of rFVIIa makes it probable that haemostatic effects will be compromised in the absence of plasma/platelet FV.…”

Section: Discussionmentioning

confidence: 85%

Management of surgical procedures in children with severe FV deficiency: experience of 13 surgeries

et al. 2012

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Homozygous severe factor V (FV) deficiency has a prevalence of around one per million. Even in patients with FV levels of <0.01 IU mL(-1) there appears to be a variation in bleeding phenotype in that there is a subgroup of affected individuals who present in later childhood and have a relatively mild bleeding phenotype, but there are children who present as neonates with intracerebral bleeding events and who have a much more severe bleeding phenotype. The only available current FV replacement is in the form of fresh frozen plasma (FFP) or solvent detergent FFP. We present here our experience with surgical haemostatic cover for 13 surgeries in three children with severe FV deficiency.

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Section: Discussionmentioning

confidence: 85%

Management of surgical procedures in children with severe FV deficiency: experience of 13 surgeries

et al. 2012

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“…A possible alternative to FFP is recombinant FVIIa [31], which is currently only approved for use in patients with FVII deficiency and patients with inhibitors (see next). However, this product is being used off‐label to treat bleeding as a result of liver disease and overdoses of warfarin.…”

Section: Managementmentioning

confidence: 99%

Factor V deficiency: a concise review

2008

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Summary. Factor V (FV; proaccelerin or labile factor) is the plasma cofactor for the prothrombinase complex that activates prothrombin to thrombin. FV deficiency can be caused by mutations in the FV gene or in genes encoding components of a putative cargo receptor that transports FV (and factor VIII) from the endoplasmic reticulum to the Golgi. Because FV is present in platelet α‐granules as well as in plasma, low FV levels are also seen in disorders of platelet granules. Additionally, acquired FV deficiencies can occur in the setting of rheumatologic disorders, malignancies, and antibiotic use and, most frequently, with the use of topical bovine thrombin. FV levels have limited correlation with the risk of bleeding, but overall, FV‐deficient patients appear to have a less severe phenotype than patients with haemophilia A or B. The most commonly reported symptoms are bleeding from mucosal surfaces and postoperative haemorrhage. However, haemarthroses and intramuscular and intracranial haemorrhages can also occur. Because no FV‐specific concentrate is available, fresh frozen plasma remains the mainstay of treatment. Antifibrinolytics can also provide benefit, especially for mucosal bleeding. In refractory cases, or for patients with inhibitors, prothrombin complex concentrates, recombinant activated FVIIa, and platelet transfusions have been successfully used. Some patients with inhibitors may also require immunosuppression.

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“…Recombinant-activated factor VII is licensed for the management of bleeding in patients with severe haemophilia and inhibitors and in treating bleeding in patients with severe platelet defects. There are reports of the off-label use of rFVIIa in FV deficient patients with and without inhibitors [3, 4]. The mode of action of rFVIIa makes it probable that haemostatic effects will be compromised in the absence of plasma/platelet FV.…”

Section: Discussionmentioning

confidence: 99%

Hip Replacement Surgery in 14-Year-Old Girl with Factor V Deficiency: Haemostatic Treatment and Thromboprophylaxis

Mingot‐Castellano

Pérez-Núñez

Baeza-Montañez

2016

Case Reports in Hematology

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Factor V (FV) is a pivotal coagulation factor present in plasma and platelets. It plays an essential role in secondary haemostasis acting as a cofactor in the prothrombinase complex, catalysing the conversion of prothrombin to thrombin. There is little evidence on the management of mayor orthopaedic surgery in paediatric or adolescents subjects with this coagulopathy and almost no information about thromboprophylaxis in these situations. We report a case of a hip replacement in a 14-year-old girl with moderate FV deficiency (0.07 IU mL−1). As haemostatic replacement, inactivated fresh frozen plasma (FFP) was transfused at doses of 600 mL (15 mL kg−1, 45 kg weight) 2 hours before surgery and then sequential FFP infusions of 250 mL (7 mL kg−1) every 12 hours for 7 days. Plasma factor VIII, von Willebrand factor antigen, and von Willebrand ristocetin cofactor were monitored to avoid supranormal levels. Since the patient was sexually mature (Marshall and Tanner stage 5) with the hormone replacement therapy, she was immobilized and the surgery was considered as a high thrombotic risk. Thus, low molecular weight heparin was administered at doses of intermediate risk (Enoxaparin 20 mg daily, by weight) after finishing the daily infusion of plasma: 24 hours and during the 7 days after intervention. No tranexamic acid was used. No haemorrhagic or thrombotic adverse event was described.

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The use of activated recombinant coagulation factor VII during haemarthroses and synovectomy in a patient with congenital severe factor V deficiency

Cited by 27 publications

References 4 publications

Management of surgical procedures in children with severe FV deficiency: experience of 13 surgeries

Management of surgical procedures in children with severe FV deficiency: experience of 13 surgeries

Factor V deficiency: a concise review

Hip Replacement Surgery in 14-Year-Old Girl with Factor V Deficiency: Haemostatic Treatment and Thromboprophylaxis

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