Sidharthan Neeraj, Sudevan Remya
1Department of Haemato Oncology, Amrita Institute of Medical Sciences, Kochi, India.
2Department of Health Sciences Research, Amrita Institute of Medical Sciences, Kochi, India.
Indian J Hematol Blood Transfus. 2020 Jan;36(1):16-25. doi: 10.1007/s12288-019-01147-0. Epub 2019 Jun 15.
Hemophilia is an inherited bleeding disorder which causes impaired blood clotting. The severity of disease depends on the type of Hemophilia, level of clotting factor concentrate (CFC), phenotypic heterogeneity and the development of inhibitors. The currently accepted standard of care of this disease is prophylaxis therapy (PT) with CFC. Prophylaxis therapy for Hemophilia is given in developed countries for the last few decades. On the contrary, episodic therapy (ET) is still the mode of treatment in middle and low income countries. ET is documented to have several potential risks such as increased bleeding rate, disability due to haemarthrosis, poor quality of life and increased chances of mortality. Several studies conducted in developed countries have confirmed the clinical efficacy of PT in Hemophilia treatment. Currently, there exist several challenges for implementation of this effective treatment in resource poor nations. Low dose prophylaxis (LDP) has been developed as a solution to minimize these challenges and to provide better care for subjects with Hemophilia from low resource countries. The impact of LDP was evaluated by several recent studies and the reported clinical outcomes seem to suggest an optimistic future for this line of therapy. Several themes related to Hemophilia care like inhibitor development, tolerance, pharmacokinetics of CFCs and cost-benefit analysis of different prophylaxis regimens are currently understood poorly. These distinct elements are highly relevant to assess the actual benefits of LDP regimen in a global scale.
血友病是一种遗传性出血性疾病,会导致血液凝固功能受损。疾病的严重程度取决于血友病的类型、凝血因子浓缩物(CFC)水平、表型异质性以及抑制剂的产生情况。目前公认的这种疾病的治疗标准是使用CFC进行预防性治疗(PT)。在过去几十年里,发达国家一直在对血友病进行预防性治疗。相反,在中低收入国家,按需治疗(ET)仍然是主要的治疗方式。据记载,按需治疗存在一些潜在风险,如出血率增加、因关节积血导致残疾、生活质量差以及死亡几率增加。发达国家进行的多项研究证实了预防性治疗在血友病治疗中的临床疗效。目前,在资源匮乏的国家实施这种有效治疗存在诸多挑战。低剂量预防性治疗(LDP)已被开发出来,以尽量减少这些挑战,并为来自资源匮乏国家的血友病患者提供更好的治疗。最近的几项研究评估了低剂量预防性治疗的效果,报告的临床结果似乎表明这种治疗方法前景乐观。目前,人们对与血友病治疗相关的几个主题,如抑制剂的产生、耐受性、CFC的药代动力学以及不同预防性治疗方案的成本效益分析了解甚少。这些不同的因素对于在全球范围内评估低剂量预防性治疗方案的实际益处高度相关。