Department of Anesthesiology and Perioperative Medicine, Tufts Medical Center, Boston, Massachusetts.
Tufts University School of Medicine, Boston, Massachusetts.
Transfusion. 2019 Jul;59(7):2234-2237. doi: 10.1111/trf.15322. Epub 2019 Apr 29.
Congenital factor V deficiency, also called parahemophilia, is a rare hematological disorder that can be treated with platelet transfusion.
A 27-year-old G2P0100 with factor V deficiency was admitted for induction of labor and requested labor epidural analgesia. Throughout her hospital course, factor V levels were managed per recommendation from her hematologist, which included transfusing fresh frozen plasma (FFP) to maintain a factor V level of 50% before any neuraxial technique and 40% for postpartum hemostasis. The parturient required multiple transfusions of FFP to stay at this level, which eventually resulted in pulmonary edema. Given the request to maintain high levels of factor V, the parturient was transfused with platelets as an alternative source of factor V. The parturient eventually delivered a healthy neonate without signs of postpartum hemorrhage or epidural hematoma.
A major learning point from this case is that platelet transfusion is an effective alternative in the management of factor V deficiency. Factor V released by platelets has enhanced procoagulant function, resulting in local factor V concentrations 100 times more than that of plasma, and has a significantly extended half-life. Platelet transfusion should be considered as a therapy in treating parturients with factor V deficiency.
先天性因子 V 缺乏症，也称为副血友病，是一种罕见的血液疾病，可以通过血小板输注进行治疗。
一位 27 岁 G2P0100 的因子 V 缺乏症患者因引产入院，并要求分娩硬膜外镇痛。在她的整个住院期间，根据她的血液病医生的建议来管理因子 V 水平，这包括在进行任何脊柱技术之前输注新鲜冷冻血浆（FFP）以将因子 V 水平维持在 50%，并在产后止血时维持在 40%。患者需要多次输注 FFP 才能保持这个水平，这最终导致了肺水肿。鉴于维持高水平因子 V 的要求，患者接受了血小板输注作为因子 V 的替代来源。最终，产妇顺利分娩，没有产后出血或硬膜外血肿的迹象。
从这个病例中得到的一个主要学习点是，血小板输注是因子 V 缺乏症管理的有效替代方法。血小板释放的因子 V 具有增强的促凝功能，导致局部因子 V 浓度比血浆高 100 倍，半衰期显著延长。血小板输注应被视为治疗因子 V 缺乏症产妇的一种疗法。
