Abstract Number: PB0721
Meeting: ISTH 2021 Congress
Background: Congenital FV deficiency is a bleeding disorder, with mild to severe hemorrhagic symptoms and low prevalence in population. Patients with significant symptoms show very low or unmeasurable plasma FV levels and are usually homozygous or compound heterozygous for mutations in FV gene. The only therapy is fresh-frozen plasma (FFP). Thromboembolic events have not been described -to our knowledge- in these patients and no guidelines are published to guide the treatment, if it were possible
Aims: Report an unusual case of severe FV Deficiency with jugular vein thrombosis after port-a-Cath insertion and our management.
Methods: Female, 78 years, severe FV deficiency (FV:C 1-2%): Multiple admissions for bleedings of variable severity.She was admitted with hematoma in left biceps femoris, starting FFP (10 ml / Kg / 12h; target trough level: 15-20%); Given the poor venous accesses, on 4th day, we requested placement of central venous catheter (Port-a-Cath). 72 hours after, she presented swelling on right neck, erythema and pain. On suspicion of thrombosis, ECODoppler was performed and showed thrombosis of right internal jugular vein without involvement of catheter.
Results: We maintain FFP (trough level FV 15-20%) and started anticoagulation: Enoxaparin®, first dose 1 mg / Kg weight followed by 0.5 mg/ Kg /12 hours (AntiXa level close to 0.5 units / ml, between therapeutic and prophylactic range and simultaneous empirical antibiotic therapy, 10 days, (not identifying germ in cultures). The treatment was carried out for 10 days, well tolerance, without adverse events.3 months after discharge, ECOdoppler showed complete resolution of thrombus.
Conclusions: It´s unusual venous thrombosis in severe FV deficiency and hemorrhagic phenotype, probably influenced by placement of central catheter, without being able to rule out associated infection, managed with minimal anticoagulation. Given the rarity, it´s interesting to report it since it is difficult to generate any scientific evidence to establish management recommendations.
To cite this abstract in AMA style:Lopez MR, Lopez Ansoar E, Nieto Vazquez A, Albo Lopez C. Thromboembolic Disease in Severe Factor V (FV) Deficiency: A Case Report: “Anticoagulating the Anticoagulable” [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/thromboembolic-disease-in-severe-factor-v-fv-deficiency-a-case-report-anticoagulating-the-anticoagulable/. Accessed November 28, 2023.
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