Abstract
Introduction. Among the most common congenital coagulopathies are haemophilia and Von Willebrand disease. These illnesses are often mimicked by orphan hereditary coagulopathies, including combined coagulation factor V and VIII deficiency.Aim — description of a clinical presentation, hampered diagnosis and choice of haemostatic therapy in a surgical patient with combined blood coagulation factor V and VIII deficiency.Main findings. We describe a clinical case of congenital combined factor V and VIII deficiency and detail the aetiology, frequency, localisation and intensity of haemorrhages. Comorbidity and surgical indications are demonstrated to require an inter-specialty medical involvement.
Highlights
Among the most common congenital coagulopathies are haemophilia and Von Willebrand disease
We describe a clinical case of congenital combined factor V and VIII deficiency and detail the aetiology, frequency, localisation and intensity of haemorrhages
Peyvandi F., Tuddenham Е., Akhtari А., et al Bleeding symptoms in 27 Iranian patients with the combined deficiency of factor V and factor VIII
Summary
Among the most common congenital coagulopathies are haemophilia and Von Willebrand disease. Цель настоящей работы — представить описание клинических проявлений, сложности диагностики заболевания и выбора гемостатической терапии при хирургических вмешательствах у больной с сочетанным дефицитом FV и FVIII. В предоперационном периоде показатели коагулограммы были следующими: АЧТВ — 92 с, протромбин по Квику — 32 %, FV — 6 %, FVIII — 6 %.
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