Bucher U, Leupin L
Folia Haematol Int Mag Klin Morphol Blutforsch. 1982;109(6):912-20.
It is essential for an efficient substitution to define the nature of the defect as good as possible. Simple screening tests allow a rapid classification. Prophylactic substitution is recommended in potentially reversible defects (bone marrow aplasia in connection with leukaemia treatment) and/or imminent bleeding (eventually complicated by additional risk factors). If bleeding cannot be stopped surgically therapeutic substitution is indicated. In case of bone marrow failure, a substitution may be particularly promising. In presence of an increased peripheral platelet destruction (disseminated intravascular coagulation, antithrombocytic antibodies) treatment of the basic disease is mandatory. Combined hemostatic defects can be influenced by fresh frozen plasma (FFP). Fresh whole blood (not older than 48 hours) may be considered in cases of thrombocytopenia and concomitant anemia. For isolated defects (e.g. hemophilias with or without antibodies, congenital afibrinogenemia, lack of factor XIII) special preparations are at hand. The clinical effect of substitution depends on the specific activity of the preparation, on the volume of expansion in the recipient and on other pharmacokinetic factors. Hepatitis and antibody-production may be considered as particularly grave side-effects.
对于有效的替代治疗而言，尽可能明确缺陷的性质至关重要。简单的筛查试验可实现快速分类。对于潜在可逆性缺陷（与白血病治疗相关的骨髓再生障碍）和/或即将发生的出血（最终可能因其他危险因素而复杂化），建议进行预防性替代治疗。如果出血无法通过手术止血，则需进行治疗性替代治疗。在骨髓衰竭的情况下，替代治疗可能特别有前景。在外周血小板破坏增加（弥散性血管内凝血、抗血小板抗体）的情况下，必须治疗基础疾病。联合止血缺陷可受新鲜冰冻血浆（FFP）影响。在血小板减少并伴有贫血的情况下，可考虑使用新鲜全血（不超过48小时）。对于孤立性缺陷（如伴有或不伴有抗体的血友病、先天性纤维蛋白原血症、缺乏因子 XIII），有专门的制剂可供使用。替代治疗的临床效果取决于制剂的比活性、受者体内的扩容体积以及其他药代动力学因素。肝炎和抗体产生可能被视为特别严重的副作用。
