Naderi Majid, Tabibian Shadi, Alizadeh Shaban, Hosseini Soudabeh, Zaker Farhad, Bamedi Taregh, Shamsizadeh Morteza, Dorgalaleh Akbar
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.
Acta Haematol. 2015;133(2):148-54. doi: 10.1159/000363598. Epub 2014 Sep 26.
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.
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.
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.
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.
因子V缺乏症（FVD）是一种罕见的出血性疾病（RBD），主要出现在近亲结婚率高的地区。在伊朗东南部的锡斯坦和俾路支斯坦（S&B），因子V缺乏症是继因子XIII缺乏症之后第二常见的RBD。本研究的目的是评估因子V缺乏症患者出血素质的临床表现和严重程度。
本描述性研究对S&B省的23例因子V缺乏症患者进行。通过临床检查和常规实验室检查诊断因子V缺乏症。根据症状严重程度将出血素质分为三个等级（I - III级）。将我们患者出血发作的严重程度与其他RBD进行比较。
根据残余血浆因子V活性，6例（26%）、16例（69.5%）和1例（4.5%）患者分别有轻度、中度和重度因子缺乏。24%的患者有III级危及生命的出血发作，与因子VII缺乏症（17.4%）和因子I缺乏症（21%）相比，发病率更高，与因子X缺乏症（41.7%）和因子XIII缺乏症（63.1%）相比，发病率更低。分别有56.2%和16.7%的患者观察到II级和I级出血素质。
因子V缺乏症是S&B省第二常见的RBD类型，II级出血发作是主要的出血表现，超过半数患者出现该情况。
