Öner Ahmet F, Celkan Tiraje, Timur Çetin, Norton Miranda, Kavaklı Kaan
Yüzüncü Yıl University Faculty of Medicine, Department of Pediatric Hematology, Van, Turkey.
İstanbul University Cerrahpaşa Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey.
Turk J Haematol. 2018 May 25;35(2):129-133. doi: 10.4274/tjh.2017.0446. Epub 2018 Mar 16.
Hereditary factor X (FX) deficiency is a rare bleeding disorder more prevalent in countries with high rates of consanguineous marriage. In a prospective, open-label, multicenter phase 3 study, 25 IU/kg plasma-derived factor X (pdFX) was administered as on-demand treatment or short-term prophylaxis for 6 months to 2 years. In Turkish subjects (n=6), 60.7% of bleeds were minor. A mean of 1.03 infusions were used to treat each bleed, and mean total dose per bleed was 25.38 IU/kg. Turkish subjects rated pdFX efficacy as excellent or good for all 84 assessable bleeds; investigators judged overall pdFX efficacy to be excellent or good for all subjects. Turkish subjects had 51 adverse events; 96% with known severity were mild/moderate, and 1 (infusion-site pain) was possibly pdFX-related. These results demonstrate that 25 IU/kg pdFX is safe and effective in this Turkish cohort (ClinicalTrials.gov identifier: NCT00930176).
遗传性因子X(FX)缺乏症是一种罕见的出血性疾病,在近亲结婚率高的国家更为普遍。在一项前瞻性、开放标签、多中心3期研究中,给予25 IU/kg血浆源性因子X(pdFX)进行按需治疗或短期预防,为期6个月至2年。在土耳其受试者(n = 6)中,60.7%的出血为轻度。每次出血平均使用1.03次输注进行治疗,每次出血的平均总剂量为25.38 IU/kg。土耳其受试者对所有84次可评估的出血将pdFX疗效评为优秀或良好;研究者对所有受试者的总体pdFX疗效判断为优秀或良好。土耳其受试者发生了51起不良事件;已知严重程度的不良事件中,96%为轻度/中度,1起(输注部位疼痛)可能与pdFX有关。这些结果表明,25 IU/kg pdFX在该土耳其队列中是安全有效的(ClinicalTrials.gov标识符:NCT00930176)。