Fresh frozen plasma in the pediatric age group and in congenital coagulation factor deficiency.

Generally, the rules of good practice in transfusion medicine apply also to the pediatric age group. However, the frequency of specific diseases that might necessitate the administration of fresh frozen plasma (FFP) differs from that in adults. Physiologic differences to the later age exist in the neonatal period and in young infants, especially with respect to the hemostatic system, that must be recognized when considering administration of FFP. The plasma levels of many procoagulant factors and important anticoagulants are lower in neonates than in other age groups. Despite these findings, healthy neonates show no easy bruising, no increased bleeding during surgery, and excellent wound healing. The same discrepancy obtains between in vitro and clinical findings with primary hemostasis in neonates. The good primary hemostasis in neonates despite poor in vitro platelet function seems to be due mainly to a very high von Willebrand factor and the presence of more high-multimeric subunits of von Willebrand factor than later in life. We must assume that these particular plasma levels of procoagulant and anticoagulant proteins are essential for the correct function of neonatal hemostasis. Evidence that the hemostatic system of neonates works best with physiologic concentrations of procoagulants and anticoagulants can also be inferred from studies where the administration of clotting factor concentrates gave poor results.Since healthy neonates and young infants have excellent hemostasis, there is absolutely no indication to 'correct' these values to adult's norms prior to invasive procedures by administering FFP. Indications for FFP, met more frequently in the pediatric age group than later in life, are exchange transfusion and extracorporeal membrane oxygenation. Indications applying equally to adults are other extracorporeal life support systems, disseminated intravascular coagulation, hepatic coagulopathy, and 'complex unclear coagulopathies'. In congenital clotting factor deficiency, replacement therapy is much more easily administered using a highly specific concentrate. When FFP is used to raise the level of the congenitally deficient factor, the huge volume needed to reach sufficiently high plasma levels can frequently be a major problem. For this reason, FFP as a replacement therapy in congenital factor deficiency is only indicated when no specific concentrate is available, as is the case in factor V deficiency and factor XI deficiency.