Acta Haematologica
Original Paper
Congenital Factor V Deficiency: Comparison of the Severity of Clinical Presentations among Patients with Rare Bleeding DisordersNaderi M.a · Tabibian S.b · Alizadeh S.b · Hosseini S.c · Zaker F.d · Bamedi T.a · Shamsizadeh M.e · Dorgalaleh A.baDepartement of Pediatric Hematology and Oncology, Ali Ebn-e Abitaleb Hospital, Research Center for Children and Adolescent Health (RCCAH), Zahedan University of Medical Sciences, Zahedan, bDepartment of Hematology, Allied Medical School, Tehran University of Medical Sciences, cDepartment of Hematology, Allied Medical School, and dDepartment of Hematology, Cellular and Molecular Research Center, School of Allied Medical Sciences, Iran University of Medical Sciences, Tehran, eSchool of Nursing and Midwifery, Shahroud University of Medical Sciences, Shahroud, Iran
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Article / Publication Details
Received: November 19, 2013
Accepted: May 07, 2014
Published online: September 26, 2014
Issue release date: February 2015
Number of Print Pages: 7
Number of Figures: 4
Number of Tables: 1
ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)
For additional information: https://www.karger.com/AHA
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. Result: 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. Conclusion: 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.
© 2014 S. Karger AG, Basel
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Article / Publication Details
Received: November 19, 2013
Accepted: May 07, 2014
Published online: September 26, 2014
Issue release date: February 2015
Number of Print Pages: 7
Number of Figures: 4
Number of Tables: 1
ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)
For additional information: https://www.karger.com/AHA
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