title: Congenital factor V deficiency: comparison of the severity of clinical presentations among patients with rare bleeding disorders.
abstract: BACKGROUND
  Factor V deficiency (FVD) is a rare bleeding disorder (RBD) mostly present in regions with a high rate of consanguinity. FVD after FXIII deficiency is the next more prevalent RBD in Sistan and Baluchistan (S&B) in southeastern Iran. The aim of this study was to evaluate the clinical manifestations and severity of bleeding diathesis in patients with FVD.
  
  
  METHODS
  This descriptive study was conducted on 23 patients with FVD in S&B province. FVD was diagnosed by clinical findings and routine laboratory tests. Bleeding diatheses were classified into three grades (I-III) depending on the severity of symptoms. The severity of bleeding episodes in our patients was compared with other RBDs.
  
  
  RESULTS
  Based on residual plasma FV activity, 6 (26%), 16 (69.5%) and 1 (4.5%) patients had mild, moderate and severe factor deficiency, respectively. 24% of the patients had grade III life-threatening bleeding episodes which in comparison with FVII deficiency (17.4%) and FI deficiency (21%) had a higher incidence, and in comparison with FX deficiency (41.7%) and FXIII deficiency (63.1) had a lower incidence. Grade II and grade I bleeding diathesis were observed in 56.2 and 16.7% of the patients, respectively.
  
  
  CONCLUSIONS
  FVD is the second most common type of RBD in S&B province and grade II bleeding episodes were the major bleeding presentation and observed in more than half of the patients.
authors.affiliation: Departement of Pediatric Hematology and Oncology, Ali Ebn-e Abitaleb Hospital, Research Center for Children and Adolescent Health (RCCAH), Zahedan University of Medical Sciences, Zahedan, Iran.
authors.forename: Majid
authors.initials: M
authors.lastname: Naderi
authors.forename: Shadi
authors.initials: S
authors.lastname: Tabibian
authors.forename: Shaban
authors.initials: S
authors.lastname: Alizadeh
authors.forename: Soudabeh
authors.initials: S
authors.lastname: Hosseini
authors.forename: Farhad
authors.initials: F
authors.lastname: Zaker
authors.forename: Taregh
authors.initials: T
authors.lastname: Bamedi
authors.forename: Morteza
authors.initials: M
authors.lastname: Shamsizadeh
authors.forename: Akbar
authors.initials: A
authors.lastname: Dorgalaleh
mesh_terms: D005166:Factor V Deficiency / Q000097:blood* / Q000453:epidemiology / Q000473:pathology*; D005260:Female; D006801:Humans; D007492:Iran / Q000453:epidemiology; D008297:Male; D012720:Severity of Illness Index