Public Health Problems related to factor V deficiency in southeast of Iran

Factor V (FV) deficiency is a rare autoso-mal recessive hemorrhagic disorder with incidence of around one per million in gen-eral populations (1,3). The disorder results from low or unmeasurable level of FV anti-gen or activity. This disorder characterized by various bleeding features including mu-cosal tract bleeding, epistaxis, oral cavity hemorrhage, menorrhagia, bruising, hema-tomas and hemarthrose. This disorder most-ly seen in some regions including Sistan and Baluchistan province in south east of Iran with high rate of consanguineous marriage. Factor V deficiency after Factor XIII defi-ciency has the highest prevalence in this province. It seems that the high frequency of the disease in the province is associated with different characteristics including eth-nic, race and religion. Thus, the aim of this study is to evaluate the role of ethical, racial and other related characteristics of the area and its relation to the high prevalence of disease in this province. Nineteen patients with FV deficiency referred to Iranian Blood Transfusion Organization (IBTO) during 2011 To 2012 were included. The deficiency in each patient established by considering clinical manifestations, family history and prolonged coagulation tests in-cluding PT and PTT and the deficiency confirmed by PT-based FV assay. Factor VIII coagulant activity measured in order to distinguished isolated FV deficiency from combined FV and FVIII deficiency. Then each patient was interviewed by an expert staff to complete a questionnaire re-garding demographic data and previous medical history, type of treatment, dura-tion, therapeutic response, clinical manifes-tations and complications of the treatment .The mean age of patients was 23.65 years. Eleven (57. 9%) patients were men and 8 (42.11%) patients were women.