Factor V and VIII combined deficiency: Clinical perioperative management for tonsillectomy in a child

Lanchon, Romain; Robin, Florian; Brissaud, O.; Marro, Matteo; Nouette‐Gaulain, Karine

doi:10.1016/j.annfar.2014.01.005

Cited by 5 publications

(3 citation statements)

References 9 publications

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“…In the STER study, all enrolled patients had FVII coagulant activity levels of ≤20% of normal. 31 In this series also to be safe, pre-operative replacement therapy was administered to patient 21 before circumcision although he was asymptomatic. In a study aiming to explore the relationship between F:C activity level and the clinical bleeding severity, it was shown that F:C activity levels that were necessary for patients to remain asymptomatic were: fibrinogen > 100 mg dl, FV 12 U dl, combined FV + VIII 43 U dl, FVII 25 U dl, FX 56 U dl, FXI 26 U dl and FXIII 31 U dl.…”

Section: Discussionmentioning

confidence: 89%

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Bleeding characteristics and management of minor surgeries in rare bleeding disorders: report from a Turkish Pediatric Hematology Center

2020

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Rare bleeding disorders including factor (F) II, FV, combined FV and FVIII, FVII, FX, FXI, FXIII, and fibrinogen deficiencies represent 3-5% of all inherited coagulation deficiencies. [1][2][3] In addition to rarity of these deficiencies, the severity of the bleeding symptoms is heterogenous. Patients may suffer from mucocutaneous bleeding, but more severe bleeding like gastrointestinal (GIS) bleeding, central nervous system (CNS) bleeding, post-traumatic hemarthrosis and hematomas, or umbilical cord bleeding may be observed. Some patients are asymptomatic, but they may present with surgical or invasive procedure-associated bleeding. 3,4 Management of surgery is based on experience from case reports. 5 Bleeding history of the patient or family may determine the severity of the disorder. Plasma F:C activity is not always correlated with bleeding severity. 6Asymptomatic patients or patients with mild symptoms undergoing minor surgery are a dilemma for hematologists although there are some reports and guidelines about management of bleeding episodes and minor surgery. 3,5,7,8

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Section: Discussionmentioning

confidence: 89%

“…In the treatment of the bleeding episodes and surgery replacement treatment with FFP, rFVIIIa and desmopressin is recommended. 31,32 FFP is a blood component that is easily available and relatively cheap. But there is still a risk of blood-borne infections, volume overload, and transfusion related acute lung injury.…”

Section: Discussionmentioning

confidence: 99%

Bleeding characteristics and management of minor surgeries in rare bleeding disorders: report from a Turkish Pediatric Hematology Center

2020

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“…Most of the surgeries reported in previous literature for F5F8D patients were lowrisk bleeding surgeries, such as tooth extraction, tonsillectomy, and circumcision. [2][3][4] Experience in managing this disease during high-risk…”

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confidence: 99%

Successful hip arthroplasty in a patient with combined deficiency of factor V and factor FVIII

Yang,

Mu,

Zhou

et al. 2023

Haemophilia

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Combined deficiency of factor V and factor VIII (F5F8D) is a rare inherited bleeding disorder. 1 Activity levels of factor V (FV) and factor VIII (FVIII) are usually between 5% and 30% of normal. F5F8D is characterized by mild to moderate bleeding symptoms such as easy bruising, epistaxis, gum bleeding, menorrhagia, and prolonged bleeding after surgery. Notably, performing surgery on patients with F5F8D has been a greater clinical challenge due to the high-risk bleeding. Most of the surgeries reported in previous literature for F5F8D patients were lowrisk bleeding surgeries, such as tooth extraction, tonsillectomy, and circumcision. [2][3][4] Experience in managing this disease during high-risk

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The use of prophylaxis in the treatment of rare bleeding disorders

Shapiro

2020

Thrombosis Research

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Factor V and VIII combined deficiency: Clinical perioperative management for tonsillectomy in a child

Cited by 5 publications

References 9 publications

Bleeding characteristics and management of minor surgeries in rare bleeding disorders: report from a Turkish Pediatric Hematology Center

Bleeding characteristics and management of minor surgeries in rare bleeding disorders: report from a Turkish Pediatric Hematology Center

Successful hip arthroplasty in a patient with combined deficiency of factor V and factor FVIII

The use of prophylaxis in the treatment of rare bleeding disorders

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