Giangrande P L F, Peyvandi F, O'Mahony B, Behr-Gross M-E, Hilger A, Schramm W, Mannucci P M
European Haemophilia Consortium, Brussels, Belgium.
University of Oxford, Oxford, UK.
Haemophilia. 2017 May;23(3):370-375. doi: 10.1111/hae.13211. Epub 2017 Apr 12.
This report summarizes recommendations relating to haemophilia therapy arising from discussions among experts from 36 European countries during the 'Kreuth IV' meeting in May 2016.
The objective of the meeting was for experts in the field of haemophilia from across Europe to draft resolutions regarding current issues relating to the treatment of haemophilia.
Hospitals providing clinical care for people with haemophilia and related disorders are strongly recommended to seek formal designation as either European Haemophilia Treatment Centres (EHTC) or European Haemophilia Comprehensive Care Centres (EHCCC). There should be agreed national protocols or guidelines on management of the ageing patient with haemophilia. The minimum consumption of factor VIII and IX concentrate in any country should be 4 IU and 0.5 IU per capita of general population respectively. Treatment for hepatitis C with direct-acting antiviral agents should be provided to all people with haemophilia on a priority basis. Genotype analysis should be offered to all patients with severe haemophilia. Genetic counselling, when given, should encompass the recommendation that genetic relatives of the affected person be advised to seek genetic counselling. People with inhibitors should have access to bypassing agents, immune tolerance and elective surgery. National or regional tenders for factor concentrates are encouraged. Outcome data including health related quality of life should be collected. Treatment with extended half-life factors should be individualized and protection against bleeding should be improved by increasing trough levels. Steps should be taken to understand and minimize the risk of inhibitor development.
It is hoped that these recommendations will help to foster equity of haemophilia care throughout Europe.
本报告总结了2016年5月在“克吕特四世”会议期间，来自36个欧洲国家的专家们就血友病治疗相关问题进行讨论后提出的建议。
本次会议的目的是让欧洲各地血友病领域的专家就当前血友病治疗相关问题起草决议。
强烈建议为血友病及相关疾病患者提供临床护理的医院申请正式指定为欧洲血友病治疗中心（EHTC）或欧洲血友病综合护理中心（EHCCC）。对于老年血友病患者的管理，应有商定的国家方案或指南。任何国家的凝血因子VIII和IX浓缩剂的最低人均消耗量应分别为4国际单位和0.5国际单位。应优先为所有血友病患者提供直接作用抗病毒药物治疗丙型肝炎。应为所有重度血友病患者提供基因分型分析。进行遗传咨询时，应建议受影响者的基因亲属寻求遗传咨询。有抑制物的患者应能获得旁路制剂、免疫耐受和择期手术。鼓励对凝血因子浓缩剂进行国家或地区招标。应收集包括健康相关生活质量在内的结果数据。延长半衰期因子的治疗应个体化，并通过提高谷浓度来改善出血防护。应采取措施了解并尽量降低抑制物形成的风险。
希望这些建议将有助于在整个欧洲促进血友病护理的公平性。
