Congenital Factor V Deficiency: Moroccans Cases
Journal Title: Macedonian Journal of Medical Sciences (MJMS) - Year 2013, Vol 6, Issue 3
Abstract
Background and Objective: The congenital factor V deficiency, known as Owren's disease or parahemophilia is a rare autosomic recessive haemorrhagic disease of coagulation, identified for the first time in Norway in 1943. In Morocco, there is no national registry including cases of congenital coagulopathy. This study highlights the rarity of inherited factor V deficiency deficiency and the importance of coagulation testing in the diagnosis of condition. Patients and Methods: We report twelve cases of factor V deficiency at our institution. Results: Of the 12 cases, seven were followed at the haematology department; the remaining five were discovered during a family survey. The average age of our patients is 16 years old [1 - 40 years], with a female dominance (Sex ratio F/M: 2). Parental consanguinity was found in three patients, bleeding family history were found in 5 patients. The concept of bleeding was found in 6 of 7 cases of patients followed, in 1 patient’s case the discovery was fortuitous. Conclusion: Inherited factor V deficiency is a rare inherited disorder. The diagnosis is mainly based on coagulation tests; however, the bleeding tendency may be severe and occurs early in life with immeasurable factor II levels.
Authors and Affiliations
Oukkache Bouchra Louai| Teaching Hospital CHI Ibn Rochd - Hematology Laboratory, Casablanca 20200, Morocco, Marielle Igala| Ibn Rochd Teaching Hospital - Hematology Laboraty, Quartier des Hopitaux , Casablanca 20200, Morocco
Keywords
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- EP ID EP8943
- DOI 10.3889/MJMS.1857-5773.2013.0293
- Views 317
- Downloads 22
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