Development of inhibitors is currently one of the most serious complications of hemophilia treatment. Typically, the propensity to develop an inhibitor is likely influenced by both genetic and non-genetic factors. Hemophilia patients with inhibitors are partially or completely refractory to traditional replacement of the deficient clotting factors and are at increased risk of bleeding as compared to patients without inhibitors. Several cases of infant hemophilia A with inhibitor have been reported in other countries, but no such patient has so far been reported in South Korea. We report two infants affected by hemophilia A with inhibitors, both of whom had bleeding episodes that were successfully treated with recombinant activated factor VII. Clinicians should remain aware of potential inhibitor development in infant hemophilia A patients and such patients should be carefully monitored.
Factor VIIa ; Hemophilia A ; Hemorrhage ; Humans ; Infant ; Korea

Intracerebral hemorrhage is a lethal stroke type with a high morbidity and mortality. Hematoma growth is one of the independent determinants of neurological and functional outcomes after intracerebral hemorrhage. Attenuation of growth is an important therapeutic strategy. Hemostatic therapeutic intervention, given ultra-early in the course of intracerebral hemorrhage, may thus improve clinical outcomes by arresting ongoing bleeding and limiting in turn the size of the hematoma. Recombinant factor VIIa is a hemostatic drug approved to treat bleeding in hemophilia or other coagulopathy; it has also been reported to arrest bleeding in nonhemophilic cases. We reviewed of the published articles specifically addressing clinical trials of recombinant factor VIIa treatment for acute intracerebral hemorrhage and evaluate the safety and feasibility of it.
Cerebral Hemorrhage* ; Factor VIIa* ; Hematoma ; Hemophilia A ; Hemorrhage ; Mortality ; Stroke

Little is known about the prophylactic use of recombinant factor VIIa (rFVIIa) in patients undergoing surgery for a bleeding aorta employing cardiopulmonary bypass. We report the successful use of rFVIIa in a patient undergoing hypothermic circulatory arrest and prolonged cardiopulmonary bypass for repair of a DeBakey type III aortic dissection.
Aorta ; Cardiopulmonary Bypass ; Factor VIIa ; Hemorrhage ; Humans ; Recombinant Proteins

BACKGROUND: Currently, the greatest challenge in hemophilia treatment is managing hemophilia patients with inhibitors. The two main bypassing agents that are used to treat hemophilia patients with inhibitors are activated prothrombin complex concentrates (APCC) and recombinant factor VIIa (rFVIIa). Hemophilia patients with inhibitors can develop bleeding episodes, that are refractory to monotherapy with either APCC or rFVIIa and thus are often difficult to manage. METHODS: This report describes a retrospective chart review of four hospitalized patients with severe hemophilia and inhibitors who were treated with sequential therapy of APCC and rFVIIa for refractory bleeding. Sequential therapy was defined as the administration of both rFVIIa and APCC within 12 h. RESULTS: In 5 episodes experienced by 4 patients with inhibitors, bleeding was not controlled by single bypass treatment, but it was controlled when two agents were sequentially administered. Sequential therapy was administered by alternating one APCC dose to 1 to 2 rFVIIa doses, with dosing intervals ranging from 3 to 6 h. All bleeding episodes were controlled within 12 to 24 h. Sequential therapy was discontinued after 2 to 5 days. No adverse clinical events, such as thrombosis, were observed. CONCLUSION: Sequential therapy with APCC and rFVIIa was efficacious without adverse events; however, attention on thrombosis is needed. In addition, a prospective clinical trial is needed to provide further evidence for this treatment.
Factor VIIa* ; Hemophilia A* ; Hemorrhage* ; Humans ; Prothrombin* ; Retrospective Studies ; Thrombosis

Factor VIIa ; Hemorrhage ; Humans ; Leukemia, Promyelocytic, Acute ; Recombinant Proteins

Recombinant activated factor VII (rFVIIa, NovoSeven (R)) was initially developed for the treatment of bleeding in patients with hemophilia having antibodies against factor VIII or IX, and factor VII deficiency. Although the precise mode of action is still elusive and there are just several hypotheses, recently case reports have suggested a role of rFVIIa in the management of intractable or life-threatening bleeding in some non-hemophilic patients who do not respond to conventional treatments. We report the successful use of rFVIIa in a pediatric patient with intractable gastrointestinal bleeding.
Antibodies ; Factor VII Deficiency ; Factor VIIa* ; Factor VIII ; Gastrointestinal Hemorrhage* ; Hemophilia A ; Hemorrhage ; Humans

PURPOSE: Congenital factor VII(FVII) deficiency is a rare bleeding disorder and surgery can cause excessive bleeding due to an extrinsic pathway problem. It can be diagnosed by increased PT and decreased FVII level in coagulation test. Symptom varies according to the level of FVII, but it is essential to prevent intraoperative excessive bleeding. METHODS: In this report, we described the orthognatic surgery experience in a mandibular prognathism patient with congenital F'VII deficiency, in which recombinant activated factor VII(rFVIIa) was used to manage the bleeding. Rsults: We could get a successful result without any complication and there was minimal intraoperative bleeding. CONCLUSION: The orthognathic surgery could therefore be safely performed in patients with congenital factor VII deficiency using rFVIIa.
Factor VII ; Factor VII Deficiency ; Factor VIIa ; Hemorrhage ; Humans ; Orthognathic Surgery ; Prognathism ; Recombinant Proteins

Among the patients with hemophilia, 10~15% have hemophilia B, and 1~3% of the hemophilia B patients develop inhibitor to factor IX clotting activity. Allergic reactions to concentrates containing factor IX (FIX) are serious complications during the treatment of hemophilia B patients with inhibitor. Although treatment with recombinant activated factor VII (FVIIa) is generally recommended in these patients, it is limited by the agent's short half-life, a lack of experience with its use in this manner and the prohibitive cost. We report here on a case of a 9-year-old boy with severe hemophilia B with inhibitor and he had a history of anaphylaxis to FIX. The patient was successfully treated with a desensitization protocol with escalating doses of FIX in addition to administering premedications.
Anaphylaxis ; Child ; Factor IX ; Factor VIIa ; Half-Life ; Hemophilia A ; Hemophilia B ; Humans ; Hypersensitivity

Coagulation involves the regulated sequence of proteolytic activation of a series of proteins to achieve appropriate and timely hemostasis in an injured vessel. In the non-pathological state, the inciting event involves exposure of circulating factor VIIa to extravascularly expressed tissue factor, which brings into motion the series of steps which results in cell based model of coagulation. In the new concepts of coagulation system, initiation, amplification and propagation steps are involved to converse of fibrinogen to fibrin. The precisely synchronized cascade of events is counter-balanced by a system of anticoagulant mechanisms. Developmental hemostasis refers to the age-related changes in the coagulation system that are most marked during neonate and childhood. An understanding of these changes in crucial to the accurate diagnosis of hemostatic abnormalities in neonate and children. This review aims to elucidate the main events within the coagulation cascade as it is currently understood to operate in vivo, and also a short review of the anticoagulants as they relate to this model. Also this paper describes the common pitfalls observed in the clinical data related to the coagulation system in neonate to children.
Anticoagulants ; Child ; Factor VIIa ; Fibrin ; Fibrinogen ; Glycosaminoglycans ; Hemostasis ; Humans ; Infant, Newborn ; Polymethacrylic Acids ; Proteins ; Thromboplastin

Hemophilia A (factor VIII deficiency) and Hemophilia B (factor IX deficiency) are the most common and serious congenital coagulation disorders. Accurate diagnosis is important and essential for effective management. A definitive diagnosis depends on factor assay to demonstrate the presence of factor VIII or factor IX. Bleeding should be treated with factor replacement therapy at the earliest moment possible, preferably within two hours from the onset of symptoms. In spite of improvements in hemophilia therapy, arthropathy remains as a significant clinical problem. Based on numerous recommendations, a major goal of hemophilia therapy is to prevent any joint disease, and prophylaxis is superior to on-demand therapy in delaying or preventing the development of hemophilic arthropathy. Prophylaxis is the administration of clotting factors at regular intervals to prevent bleeding. Currently the most commonly suggested protocol for prophylaxis is the infusion of 25~40 IU/kg of clotting factor concentrates three times a week for those with hemophilia A and twice a week for those with hemophilia B. The management of patients who have inhibitory antibodies against factor VIII or IX remains challenging. About 10~15% of hemophilia A patients and 1~3% of hemophilia B patients may develop persistent inhibitors rendering treatments with factor concentrates difficult. Alternative agents for hemophilia inhibitor patients include bypassing agents, such as recom-binant factor VIIa and prothrombin complex concentrates. Ultimately, immune tolerance induction to eradicate the inhibitor is desired.
Antibodies ; Blood Coagulation Factors ; Factor IX ; Factor VIIa ; Factor VIII ; Hemophilia A ; Hemophilia B ; Hemorrhage ; Humans ; Immune Tolerance ; Joint Diseases ; Prothrombin