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

(4 citation statements)

References 4 publications

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“…FV-deficient patients have low plasma levels of the anticoagulant protein tissue factor pathway inhibitor, which considerably reduces the FV requirement for thrombin generation [ 1 ], so it is thought that FⅤ in platelet α-granules alone can produce sufficient amounts of thrombin for hemostasis [ 1 , 11 , 12 ]. Some reports suggest that the severity of bleeding depends on the FⅤ levels in platelets [ 2 ]. Thus, coagulation factors and platelets play an important role in the perioperative management of patients with FⅤ deficiency.…”

Section: Discussionmentioning

confidence: 99%

“…Most patients with this disorder present with mild to moderate bleeding symptoms and rarely require transfusion of blood products or hemostatic intervention [ 1 ]. However, under highly invasive circumstances such as trauma or surgical procedures (including tooth extraction), even patients with mild bleeding symptoms may be susceptible to abnormal bleeding [ 2 , 3 ], and FⅤ activity should be maintained above 15%-25% in the perioperative period [ 4 , 5 ]. In Japan, where recombinant FⅤ products are currently unavailable, fresh frozen plasma (FFP) has been used when supplementation is required [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

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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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Reports on cases of factor Ⅴ (FⅤ) deficiency complicated by platelet function disorders in patients undergoing cardiac surgery are rare, and the utilization of thromboelastography in such cases is limited. This case presents a unique case of FⅤ deficiency complicated by platelet function disorders, highlighting the significance of tailored transfusion strategies guided by thromboelastography (TEG). A 64-year-old hemodialysis patient who was diagnosed with FⅤ deficiency 24 years prior presented for an on-pump coronary artery bypass graft. The decrease in FⅤ activity on preoperative examination was mild. Based on this finding, it was determined that preoperative fresh frozen plasma supplementation was not required. However, the case was complicated by platelet function disorders; therefore, a preoperative transfusion of platelet concentrate was performed to correct the decreased platelet function, enabling subsequent surgery. Intraoperative and postoperative transfusion strategies were guided by TEG. This study highlights TEG-guided transfusion management as a viable option for patients with FⅤ deficiency complicated by platelet function disorders.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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“…Transfusion of FFP and maintenance of factor level between 15% and 20% throughout the perioperative period is imperative to prevent hemorrhagic accidents during surgical procedures. [ 14 ] Postoperative daily screening for factor level is advisable for moderation of FFP transfusion and surveillance. The management strategy employed in our case involved transfusion of FFP to proactively address the deficiency and elevate the factor V level above 20%, followed by administration of another additional unit of FFP 1 h before surgery to further bolster the factor levels and reduce the likelihood of intraoperative bleeding.…”

Section: Discussionmentioning

confidence: 99%

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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“…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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Congenital Factor V deficiency: perioperative management (case report)

Cited by 3 publications

References 4 publications

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

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

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

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

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