St Thomas' Hospital Haemophilia Reference Centre, London, UK.
Thromb Res. 2009 Dec;124 Suppl 2:S12-6. doi: 10.1016/S0049-3848(09)70159-8.
Congenital abnormalities of fibrinogen are rare disorders classified as quantitative (afibrinogenemia and hypofibrinogenemia) or qualitative types (dysfibrinogenemia and hypodysfibrinogenemia). Fibrinogen is essential to haemostasis as the substrate for fibrin clot formation and also acts in primary haemostasis as a key ligand in platelet aggregation. Quantitative deficiency of fibrinogen can result in severe bleeding, or arterial and venous thromboembolism, and poor wound healing. Dysfibrinogenemia is characterized by functional abnormalities of fibrinogen, which may be asymptomatic (in 50% of cases), or cause bleeding (25%) or thrombosis (25%). Replacement of the deficient or abnormal fibrinogen with frozen plasma, cryoprecipitate, or fibrinogen concentrate has been found to be effective in practice in treating haemostatic complications of these disorders. Although cryoprecipitate is the most commonly used replacement material, pathogen-reduced fibrinogen concentrates have several advantages, most importantly a lower potential risk of viral transmission and standardized fibrinogen content allowing accurate dosing. They also avoid transfusing unwanted clotting factors, platelet microparticles and immunoglobulins, and can be administered rapidly without thawing. The use of fibrinogen concentrate to treat congenital fibrinogen disorders is strongly supported in principle and increasingly by practical experience and evidence.
纤维蛋白原先天性异常是罕见的疾病，可分为定量（无纤维蛋白原血症和低纤维蛋白原血症）或定性类型（异常纤维蛋白原血症和低异常纤维蛋白原血症）。纤维蛋白原是止血所必需的，作为纤维蛋白凝块形成的底物，也作为血小板聚集的关键配体在初级止血中发挥作用。纤维蛋白原的定量缺乏可导致严重出血或动静脉血栓形成和伤口愈合不良。异常纤维蛋白原血症的特征是纤维蛋白原的功能异常，可能无症状（占 50%），或导致出血（25%）或血栓形成（25%）。用冷冻血浆、冷沉淀或纤维蛋白原浓缩物替代缺乏或异常的纤维蛋白原已被证明在治疗这些疾病的止血并发症方面是有效的。虽然冷沉淀是最常用的替代物，但病毒减少的纤维蛋白原浓缩物具有几个优点，最重要的是降低了病毒传播的潜在风险，以及标准化的纤维蛋白原含量允许准确给药。它们还避免输注不需要的凝血因子、血小板微粒体和免疫球蛋白，并可在不解冻的情况下快速给药。纤维蛋白原浓缩物治疗先天性纤维蛋白原疾病在原则上得到了强烈支持，并越来越多地得到实际经验和证据的支持。
