Immuno-Hematology and Blood Transfusion Department, Faculty of Medicine, Damascus University, Damascus, Syria.
Haemophilia. 2012 Nov;18(6):851-4. doi: 10.1111/j.1365-2516.2012.02862.x. Epub 2012 Jun 4.
Creating a national registry for bleeding disorders is a major step in establishing a National Hemophilia Care Program in all countries. Creating such a registry which would contain accurate and regularly updated data, including laboratory analysis confirmed by a reference laboratory established at the Syrian Hemophilia Society. Blood samples were drawn and analysed in the Society reference laboratory for the following screening tests: prothrombin time (PT), APTT and coagulation factor assays. Inhibitor detection and VWF RiCof were performed depending on the result of the screening tests. HBs Ag, anti-HCV, anti-HIV 1+2 and syphilis tests were also performed to detect transfusion transmitted agents (TTA). Diagnosis of the bleeding disorder type was confirmed for 760 of these cases. Among the 760 confirmed patients, 82.5% had haemophilia. Among these, 89.6%were haemophilia A; 10.4% were haemophilia B; 8.3% had VWD; 9.2% had other rare bleeding disorders as follows: 1.2% FVII deficiency, 0.7% FV deficiency, 1.8% F1 deficiency, 0.4% FX deficiency, 1.4% platelets dysfunctions (mainly Glanzmann Thrombasthenia) and 3.7% had combined FVIII and FV deficiency. Eighty (21.3%) cases of 375 screened for transfusion transmitted agents were positive for at least one infection: 0.5% were HBsAg positive, 19.7% were anti-HCV positive, 0.8% had combined HBsAg and anti-HCV positivity and 0.3% was anti-Syphilis positive. All patients were negative for HIV1 and HIV2. The preliminary data presented here follow known data on haemophilia A, haemophilia B and VWD disease. This registry will certainly help in improving haemophilia care in Syria.
建立国家出血性疾病登记处是在所有国家建立国家血友病护理计划的重要步骤。建立这样一个登记处，其中将包含准确和定期更新的数据，包括由叙利亚血友病协会设立的参考实验室确认的实验室分析。在协会参考实验室中抽取和分析血液样本，进行以下筛选测试：凝血酶原时间 (PT)、APTT 和凝血因子测定。根据筛选测试的结果进行抑制剂检测和 VWF RiCof。还进行 HBsAg、抗 HCV、抗 HIV1+2 和梅毒检测，以检测输血传播的病原体 (TTA)。对其中 760 例进行了出血性疾病类型的诊断确认。在这 760 例确诊患者中，82.5%患有血友病。其中，89.6%为血友病 A；10.4%为血友病 B；8.3%为血管性血友病；9.2%为其他罕见出血性疾病，如下：1.2%为 FVII 缺乏症，0.7%为 FV 缺乏症，1.8%为 F1 缺乏症，0.4%为 FX 缺乏症，1.4%为血小板功能障碍（主要为 Glanzmann 血小板病），3.7%为 FVIII 和 FV 联合缺乏症。对 375 例筛查输血传播病原体的患者中，有 80 例（21.3%）至少有一种感染呈阳性：0.5%为 HBsAg 阳性，19.7%为抗 HCV 阳性，0.8%为 HBsAg 和抗 HCV 同时阳性，0.3%为抗梅毒阳性。所有患者 HIV1 和 HIV2 均为阴性。这里呈现的初步数据遵循已知的血友病 A、血友病 B 和血管性血友病疾病数据。该登记处肯定会有助于改善叙利亚的血友病护理。
