Department of Hematology, Jagiellonian University School of Medicine, Krakow, Poland.
Haemophilia. 2011 Jan;17(1):e189-95. doi: 10.1111/j.1365-2516.2010.02371.x.
We present data collected in HemoRec, an Internet-based platform implemented in 2006 in 15 haemophilia treatment centres in Poland and compare them with the national registry of inherited bleeding disorders established since 1991 at the Institute of Haematology and Blood Transfusion in Warsaw. We also analyse the current status of haemophilia treatment in Poland as well as future perspectives. Data on 1102 patients registered in HemoRec were analysed and compared with 4294 patients in the national registry (status as at 17.08.2009). The number of patients with severe haemophilia, mild/moderate haemophilia and von Willebrand in HemoRec is 530, 328 and 54 (respectively), compared with 1199, 1167 and 1128 in the national registry. The mean age of all haemophilic patients registered in HemoRec is 26.2 years, compared with 37.3 years in the general Polish male population in 2008. The number of haemophilic patients with inhibitor registered in HemoRec is 102 compared with 155 in the national registry (resulting in a prevalence of 14.9% of all severe haemophilia A and 1.6% of all severe haemophilia B patients). HemoRec includes data on a representative group of Polish haemophilic patients, mostly with haemophilia and haemophilia with inhibitor. von Willebrand's disease is largely under-registered in Poland. The survival of Polish haemophilic patients is shorter than that in the general population. The number of inhibitor patients in Poland is relatively large and should be decreased by wider availability of immunotolerance induction in 2010.
我们呈现了在 HemoRec 中收集的数据，这是一个基于互联网的平台，于 2006 年在波兰的 15 个血友病治疗中心实施，并将其与自 1991 年以来在华沙血液学和输血研究所建立的国家遗传性出血性疾病登记处进行了比较。我们还分析了波兰目前的血友病治疗状况和未来的展望。分析了在 HemoRec 登记的 1102 名患者的数据，并与国家登记处的 4294 名患者（截至 2009 年 8 月 17 日的情况）进行了比较。在 HemoRec 中，重型血友病、轻型/中型血友病和血管性血友病患者的数量分别为 530、328 和 54，而在国家登记处分别为 1199、1167 和 1128。在 HemoRec 中登记的所有血友病患者的平均年龄为 26.2 岁，而 2008 年波兰普通男性人口的平均年龄为 37.3 岁。在 HemoRec 中登记的有抑制剂的血友病患者数量为 102 例，而在国家登记处为 155 例（导致所有重型血友病 A 患者的 14.9%和所有重型血友病 B 患者的 1.6%有抑制剂）。HemoRec 包括了波兰代表性血友病患者的数据，这些患者主要患有血友病和血友病伴抑制剂。血管性血友病在波兰的登记情况很大程度上不足。波兰血友病患者的存活率短于普通人群。波兰的抑制剂患者数量相对较多，应该通过 2010 年更广泛地提供免疫耐受诱导来减少。
