Congenital factor X deficiency: spectrum of bleeding symptoms in 32 Iranian patients |
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Authors: | Peyvandi,Mannucci,Lak,Abdoullahi,Zeinali,Sharifian,& Perry |
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Affiliation: | Haemophilia Centre, Imam Khomeini Hospital, Tehran, Iran,;Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, IRCCS Maggiore Hospital, University of Milan, Italy,;Pasteur Institute, Tehran, Iran,;Haemophilia Centre and Haemostasis Unit, Royal Free Hospital, London, U.K. |
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Abstract: | The spectrum of the clinical manifestations of congenital factor X deficiency was studied in 32 Iranian patients. The most frequent symptom was epistaxis, which occurred in 72% of patients, with all degrees of deficiency. Other mucosal haemorrhages (e.g. haematuria, gastrointestinal bleeding) were less frequent and occurred mainly in patients with unmeasurable factor X. Menorrhagia occurred in half of the women of reproductive age. Soft tissue bleeding occurred in two-thirds of the patients; spontaneous haematomas and haemarthroses led to severe arthropathy in five patients. Bleeding from the umbilical stump was an unexpected finding in nine patients. This study demonstrated that the bleeding tendency of factor X deficiency is severe and correlates with factor levels. |
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Keywords: | factor X factor X deficiency |
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