Prevention of bleeding and hemorrhagic complications in surgical patients with inherited factor VII deficiency

Abstract

Inherited factor VII (FVII) deficiency is a rare autosomal recessive hemorrhagic disorder. The major clinical symptoms include: bleeding from the oral cavity, epistaxis, menorrhagia, spontaneous hemarthros, bleeding to the gastrointestinal tract and central nervous system, and perioperative bleeding. The aim of this study was to present our experience in preventing bleeding and hemorrhagic disorders in surgical patients with inherited FVII deficiency by using recombinant activated FVIIa (rFVIIa), and with prothrombin complex concentrates (PCCs). In 2002-2011, 17 patients with inherited FVII deficiency underwent surgery. Thirteen patients had isolated FVII deficiency below 10%, and four patients 10-25. To prevent bleeding and hemorrhagic complications, we administered small single doses of rFVIIa (Novo-Seven) at 12-h intervals to 15 patients on surgery day and on day 1 following surgery, then every 24 h; PCCs were administered (Prothromplex, Beriplex) to two patients. No symptoms of bleeding, hemorrhagic or thromboembolic complications were observed in the perioperative and 1-month observation period in surgical patients treated with rFVIIa. One patient treated with PCC (Prothromplex) developed distal deep vein thrombosis on postoperative day 7. The results suggest that small, single, every 12-h doses of rFVIIa (NovoSeven) and in next days after surgery one time every 24 h are well tolerated and effective for prevention of thromboembolic, bleeding and hemorrhagic complications in FVII-deficient patients. Antithrombotic prophylaxis with low-molecular-weight heparin should be applied in patients using PCCs.

A Cross-Sectional Study of Epistaxis: Etiologic and Clinical Characteristics

“…Another group of diseases that has prevalent epistaxis attacks is clotting factor deficiencies. Epistaxis attacks, bleeding from mucosal surfaces (such as gastrointestinal bleeding), and related complications are seen in 60% of these patients [14]. Factor deficiencies were detected in only three (0.5%) patients in the present study group.…”

Management of Patients with Coagulation Disorders Undergoing Minor Oral Surgery

“…The hemorrhagic risk for these patients is considered very high when there are plasma concentrations of FVII lower than 1%, high if they are between 1% and 10%, medium if they are between 10% and 25%, and low if they are less than 25%. 6,15 For patients with other coagulation disorders for which a specific substitute is not available as for FVII deficiency, we have adopted a protocol based on antifibrinolytic agents such as tranexamic acid since its effectiveness in decreasing bleeding has been proven intra and post operative, and above all because it can be administered orally, intravenously and as a mouthwash. By adopting a protocol combined with tranexamic acid, placing a fibrin sponge inside the alveolus and closing by primary intention by means of non-resorbable intraoral sutures, we were able to achieve excellent postoperative bleeding control and minimize complications.…”

“…A severe deficiency is expressed in homozygous individuals, meanwhile heterozygous are affected by a moderate one. 5,6 The Factor V deficiency, also called Owren's disease, is a rare (1/1.000.000) autosomal recessive disorder, it affects men and women equally. Common symptoms are: bruising, nose and mouth bleeding, prolonged bleeding after trauma or surgery and spontaneous bleeding under the skin.…”

“…The purpose of the present study was to analyze the management of multiple dental extractions in patients affected by the following coagulation disorders: Factor VII deficit, Haemophilia A and Glanzmann thrombasthenia, in order to prevent bleeding intraoperative and postoperative complications. 4,6 2. Materials and Methods…”

Coagulation Factor VII: Genetic, Molecular, and Clinical Characteristics

“…Activated Recombinant Factor VII (rFVIIa) is used to treat bleeding events in patients with FVII deficiency and prevent bleeding in patients under surgery (75). Prophylactic administration of rFVIIa has been previously scheduled for children with severe congenital FVII deficiency (76).…”