Eid Suhair S, Kamal Nazmi R, Shubeilat Taisir S, Wael Abu-Ghoush Mohammed
Coagulation Section-Haematology Department, Princess Iman Research and Laboratory Sciences Center, King Hussein Medical Center, Amman, Jordan.
Clin Lab Sci. 2008 Fall;21(4):210-4.
Congenital bleeding disorders comprise a heterogeneous group of diseases that reflect abnormalities of blood vessels, coagulation proteins, and platelets. A 14-year retrospective study (1991-2005) was conducted for patients referred to the coagulation section of the Hematology Department (King Hussein Medical Center, Amman, Jordan), who had suffered from bleeding tendencies to assess the prevalence of bleeding disorders among Jordanians and to describe their clinical manifestations. Four hundred and three patients matched our criteria. All patients were screened with routine coagulation assays and a complete blood cell count; a factor assay was performed if indicated by the results of the screening assays. A total of 168 patients (41.6%) were diagnosed with a bleeding disorder caused by a factor deficiency, of which 17.1% were described as hemophilia A (n=69), 6.2% were described as vWD (n=25), and 4.2% were described as hemophilia B (n=17). A subset of the total patient population comprising 14.1% of the patients were diagnosed with a Rare Inherited Coagulation Deficiency (RICD), where 4.0% were FX deficient (n=16), 3.7% were FVII deficient (n=15), 3.7% were FV deficient (n=15), 2.5% were FXI deficient (n=10), and 0.2% were diagnosed with afibrinogenemia (n=1).
先天性出血性疾病是一组异质性疾病,反映了血管、凝血蛋白和血小板的异常。对转诊至血液科凝血科(约旦安曼侯赛因国王医疗中心)的有出血倾向的患者进行了一项为期14年的回顾性研究(1991 - 2005年),以评估约旦人出血性疾病的患病率并描述其临床表现。403名患者符合我们的标准。所有患者均接受了常规凝血检测和全血细胞计数;如果筛查检测结果表明有必要,则进行因子检测。共有168名患者(41.6%)被诊断患有由因子缺乏引起的出血性疾病,其中17.1%被描述为甲型血友病(n = 69),6.2%被描述为血管性血友病(vWD,n = 25),4.2%被描述为乙型血友病(n = 17)。占总患者人群14.1%的一个亚组被诊断患有罕见遗传性凝血缺陷(RICD),其中4.0%为因子X缺乏(n = 16),3.7%为因子VII缺乏(n = 15),3.7%为因子V缺乏(n = 15),2.5%为因子XI缺乏(n = 10),0.2%被诊断为无纤维蛋白原血症(n = 1)。