血友病的临床治疗：第55届美国血液学会年会报道  

The hemophilias and their clinical management： reports in the 55th ASH annual meeting

作　　者：胡晓静[1] 侯军[1] 施菊妹[1] 

机构地区：[1]上海市第十人民医院同济大学附属第十人民医院血液科,200072

出　　处：《白血病．淋巴瘤》2014年第2期75-78,共4页Journal of Leukemia & Lymphoma

摘　　要：血友病患者的预后在过去50年中有了显著提高.随着目前越来越多安全的凝血因子浓缩物的出现,血友病临床治疗的主要焦点成为预防长期并发症,尤其是在重型血友病患者中应预防可致残的关节病.对于有少量出血史、重型血友病A的幼年患者,早期预防性凝血因子输注治疗可取得确切的临床疗效.对于已存在关节病的青少年和成年血友病患者,可从预防性治疗中获益.凝血Ⅷ因子抑制物的产生是凝血因子替代治疗最棘手的并发症,临床上可采用抑制物免疫耐受诱导疗法来克服抑制物的作用.文章介绍了2013年第55届美国血液学会（ASH）年会关于临床治疗儿童及成年人血友病的研究进展.Outcomes for patients with hemophilia have improved dramatically over the past 50 years. With the increased availability of safe clotting factor concentrates, the primary focus in clinical, management is now the prevention of long-term complications, most notably the debilitating hemophilic arthropathy that is associated with severe disease. Definitive evidence of improved clinical results from primary prophylaxis started in youn6 patients with severe hemophilia A and a minimal bleeding history is presented. Furthermore, recent studies showing benefits for initiating prophylaxis in older adolescents and adults with established joint disease are examined. Inbibitors to factor Ⅷ are the most problematic complication of factor replacement therapy. Patient-specific and treatment-related factors that contribute to the risk of inhibitor formation are discussed and controversies and clinical evidence related to approaches for tolerance induction are reviewed. Immune tolerance induction is the proven method for eradication of inhibitors. This article reviews latest research presented at the 55th ASH annual meeting on the hemophilias and their clinical management.

关 键 词：血友病 治疗 美国血液学会年会 

分 类 号：R554.1[医药卫生—血液循环系统疾病]