看懂化验单-血友病.pptVIP

血源性凝血因子VIII Recombinate ReFacto Kogenate（拜科奇） Advate 凝血因子直接来源于人体血浆 生产过程中多个步骤使用血液成分添加剂 细胞培养阶段使用血液成分添加剂 首批在任何阶段都未使用血源性添加剂的rFVIII产品问世 1980s Early 1990s Late 1990s 2003 对安全的需求促使甲型血友病治疗手段不断进步 早期含血液成分的 重组凝血因子VIII 不含血液成分的 重组凝血因子VIII 2021/3/17 * 中国已上市/准备上市的凝血因子Ⅷ产品 血源性 凝血因子VIII 含血的 重组凝血因子VIII 不含血的 重组凝血因子VIII 海莫莱士（国产） 康斯平（国产） 绿十字（国产） 拜科奇（Kogenate FS）） Recombinate（注册中） ADVATE（注册中） Xyntha（注册中- 非全长链） 含血的凝血因子VIII 不含血的凝血因子VIII 2021/3/17 * 种类 优点 缺点 新鲜冰冻血浆 含各种凝血因子 输注量太大，造成循环负担。可能带有感染源 冷沉淀 含因子Ⅷ、vWF 可能带有感染源 浓缩因子Ⅷ、Ⅸ 纯度高，容易保存及方便携带 可能带有感染源 基因工程制造因子Ⅷ、Ⅸ 避免病毒的感染，同时不受血浆来源短缺的限制可大量制造。 价格昂贵 血友病治疗相关凝血因子的比较 2021/3/17 * * HBV was identified and documented in the mid to late 1960s.10 This discovery would unfortunately become particularly meaningful for patients with hemophilia in the next decade Beginning in the 1970s, thousands of plasma donations were being pooled in plasma concentrates, and high-potency dried replacement factors made home infusion possible.9,11 The first licensed antihemophilic factor (AHF) was Hemofil?.12 The development of plasma concentrates revolutionized the treatment of hemophilia and permitted many patients a nearly normal lifestyle9 Unfortunately, the pooling of large numbers of plasma donations for plasma concentrates of FVIII had a terrible consequence. By the late 1970s, 75% to 95% of patients with hemophilia were HBV-positive.13 In addition, non-A–non-B hepatitis, later identified as hepatitis C, was also present in a majority of patients9 In 1982, HIV was identified for the first time in 3 hemophilia patients. By the mid to late 1980s, more than three fourths of patients with hemophilia were HIV positive,9 and in the early 1990s, the vast majority of patients with hemophilia were infected with HCV14 The first in a long series of innovations to reduce contamination in FVIII concentrates began in the mid and late 1980s. Heat-treated plasma concentrates were introduced in 1984, followed by enhanced viral inactivation techniques and expanded donor screening in the