Congenital Factor V deficiency: perioperative management (case report)

Fehdi, Mohamed Anass; Lazraq, Mohamed; Benhamza, Sabah; Bensaid, A.; Miloudi, Youssef; Harrar, N. El

doi:10.11604/pamj.2020.36.46.23561

Cited by 3 publications

(1 citation statement)

References 4 publications

(11 reference statements)

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“…Upon FFP transfusion, symptoms are milder and more asymptomatic than those in case of hemophilia; the risk of death associated with bleeding events is low, and the prognosis is good [ 7 ]. There are multiple reports of FFP transfusion for surgeries, such as heart surgery [ 8 ], cochlear implant surgery [ 9 ], intracranial drainage for chronic subdural hematoma [ 10 ], laparoscopic-assisted vaginal hysterectomy, bilateral salpingo-oophorectomy [ 11 ], and laparoscopic cholecystectomy [ 12 ]. In this case, surgery or RFA was considered for treatment based on the BCLC staging algorithm [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

Laparoscopic hepatectomy for hepatocellular carcinoma in a patient with congenital factor V deficiency: a case report

et al. 2022

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Background Factor V (FV) deficiency is an extremely rare disease, with an incidence of 1 in 1 million. The bleeding symptoms are mild, and the prognosis is good; however, the safety of surgical treatment is unclear, because there are few available reports. Herein, we report a case of hepatocellular carcinoma with congenital FV deficiency in a patient who safely underwent laparoscopic hepatectomy. Case presentation A 79-year-old man, diagnosed with hepatocellular carcinoma of liver segment 5, with type C cirrhosis and sustained virological response visited our hospital. He had congenital FV deficiency, and blood tests showed coagulation deficiencies with an FV activity of < 2.6%, prothrombin time activity of 11%, and activated partial thromboplastin time of 100.3 s. Surgery and radiofrequency ablation were considered for treatment. Since the tumor was in contact with the Glissonean pedicle 5 + 6, surgery was judged to be superior from the viewpoint of safety and curability. After discussing the safety of the surgery with a hematologist, it was determined that the operation could be performed safely by transfusing sufficient fresh frozen plasma (FFP). Laparoscopic hepatic segment 5 + 6 subsegmental resection was performed with FFP transfusion, fluid restriction, airway pressure control, and central venous pressure reduction to control the bleeding. Bleeding was minimized during the transection of the liver parenchyma and no bleeding tendency was observed. The operative time was 445 min, and the amount of intraoperative bleeding was 171 mL. No complications, such as postoperative bleeding, were observed, and the patient was discharged on the eighth postoperative day. Conclusions Liver surgery can be performed safely in FV-deficient patients with strict coagulation capacity monitoring and appropriate transfusion of FFP. Preoperative evaluation of cardiac function to determine tolerance to high doses of FFP and ingenuity of surgery and intraoperative management to minimize blood loss are important.

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Section: Discussionmentioning

confidence: 99%

Laparoscopic hepatectomy for hepatocellular carcinoma in a patient with congenital factor V deficiency: a case report

et al. 2022

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Rare Coagulopathies in Hematologic Spotlight: Isolated Factor V Deficiency and Combined Factor V and VIII Deficiency

Gangadaran,

Sekar,

Rangarajan

et al. 2024

International Journal of Applied &Amp; Basic Medical Research

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Rare coagulation disorders pose significant diagnostic challenges emphasizing the importance of clinical vigilance and meticulous hemostatic workup for accurate diagnosis and timely management. We present two cases of exceptionally uncommon coagulopathies – isolated factor V deficiency (F5D) and combined factor V and VIII deficiency (F5F8D). Case 1 features a 24-year-old woman incidentally diagnosed with severe F5D during routine preoperative evaluation for an ovarian cyst. Despite the absence of any reported bleeding manifestations, a timely and accurate diagnosis was rendered. Perioperative management with fresh frozen plasma and postoperative monitoring ensured favorable surgical outcomes. Case 2 features a 10-year-old male presenting with prolonged gum bleeding. Following systematic hemostatic workup, a diagnosis of F5F8D was rendered, thereby guiding optimal therapeutic interventions. We herein aim to contribute valuable insights into the understanding of coagulation physiology and the diagnostic intricacies and management strategies of rare coagulation disorders.

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Use of Thromboelastography in Coronary Artery Bypass Grafting in a Patient With Factor Ⅴ Deficiency With Platelet Function Disorders: A Case Report and Literature Review

Kawazoe,

Tamura,

Sato

et al. 2024

Cureus

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Congenital Factor V deficiency: perioperative management (case report)

Cited by 3 publications

References 4 publications

Laparoscopic hepatectomy for hepatocellular carcinoma in a patient with congenital factor V deficiency: a case report

Laparoscopic hepatectomy for hepatocellular carcinoma in a patient with congenital factor V deficiency: a case report

Rare Coagulopathies in Hematologic Spotlight: Isolated Factor V Deficiency and Combined Factor V and VIII Deficiency

Use of Thromboelastography in Coronary Artery Bypass Grafting in a Patient With Factor Ⅴ Deficiency With Platelet Function Disorders: A Case Report and Literature Review

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