Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening disorder due to hyperinflammation resulting in infiltration of different organs with extensive hemophagocytosis. Severe coagulopathy was one of the main reasons for death in HLH. Over secretion of plasminogen activator by activated macrophages leads to hyperfibrinolysis. We reported a 36-year-old woman who was diagnosed as HLH probably secondary to lymphoma. Massive bleeding from gut and retroperitoneal area were not able to be controlled by conventional hemostatic treatments. This patient received one dose recombinant activated factor VII (rFVIIa) 3.6 mg (70 μg/kg). Hemostatic effect was achieved in 0.5 hour and lasted 24 hours. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were quickly corrected to normal ranges. Fibrinogen level elevated from 0.5 g/L before using rFVIIa to 1.8 g/L 20 hours after. Although dexamethasone and etopside were administrated to treat HLH, this patient died from septic shock after persistent neutropenia. This suggests that rFVIIa may be effective in the management of intractable hemorrhage in patients with HLH.
Adult ; Disseminated Intravascular Coagulation ; complications ; Factor VIIa ; therapeutic use ; Female ; Humans ; Lymphohistiocytosis, Hemophagocytic ; drug therapy ; Recombinant Proteins ; therapeutic use

Recombinant activated factor VII (rFVIIa) is a novel therapeutic agent for life-threatening massive gastrointestinal bleeding. We report a case of massive gastrointestinal bleeding in a 78-year-old female patient with respiratory and renal failure. After failure of management of the bleeding with routine pharmacotherapy, we gave the patient rFVIIa injection at the dose of 20 µg/kg and the bleeding was rapidly controlled. Adverse side effects of the drug were not observed in this patient.
Aged ; Factor VIIa ; therapeutic use ; Female ; Gastrointestinal Hemorrhage ; drug therapy ; Humans ; Recombinant Proteins ; therapeutic use ; Renal Insufficiency ; Respiratory Insufficiency

Definite therapeutic effect has obtained by TCM in treating acute cerebral hemorrhage (ACH) according the TCM theory of "blood circulating outside the vessels is the stasis" using breaking stagnant and eliminating blood stasis (Poxue Zhuyu) method, but no material involving the natural development of stoke in superacue stage (0 - 4 hrs after onset of the disease) being presented so far. It has been proved by randomized, double-blinded multi-centeric clinical trials that recombinant activated factor VII (rF VII a) could decreased the morbidity and disability of patients suffered from ACH, suggesting that use hemostasis treatment in ACH during superacu stage should be stressed, and the drugs for Poxue Zhuyu should be used cautiously in the period of 0 - 4 hrs after onset. The hemorrhagic disorder could be eliminated by using drugs for Poxue Zhuyu and other medicines in rational combination.
Cerebral Hemorrhage ; drug therapy ; Drugs, Chinese Herbal ; therapeutic use ; Factor VIIa ; biosynthesis ; genetics ; therapeutic use ; Humans ; Medicine, Chinese Traditional ; Phytotherapy ; Recombinant Proteins ; biosynthesis ; genetics ; therapeutic use

Acquired hemophilia A (AHA) is a rare coagulopathy caused by autoantibodies to coagulation factor VIII (FVIII). Most patients with AHA have been previously healthy; however, a variety of morbidities have been associated with the condition including pregnancy. A 40-yr-old woman visited our institution with extensive hematoma on the right hip area. Her medical history revealed no personal or familial history of bleeding diathesis. Her coagulation tests showed markedly prolonged aPTT (117 sec), markedly decreased level of FVIII activity (0.4%) and high-titer FVIII inhibitor (77 BU). Collectively, she was diagnosed as having postpartum AHA and was treated with bypassing agents and corticosteroids. Her aPTT was normalized on the 174th postpartum day and FVIII inhibitor showed negative conversion on the 224th postpartum day. This is the first case of postpartum AHA with high-titer FVIII inhibitor in Korea. Timely diagnosis and management can reduce morbidity and mortality of this potentially life-threatening condition.
Adrenal Cortex Hormones/therapeutic use ; Adult ; Autoantibodies/blood ; Blood Coagulation Factors/therapeutic use ; Factor VIII/immunology ; Factor VIIa/therapeutic use ; Female ; Hematoma/diagnosis ; Hemophilia A/*diagnosis/therapy ; Humans ; Partial Thromboplastin Time ; Postpartum Period ; Pregnancy ; Recombinant Proteins/therapeutic use ; Republic of Korea

OBJECTIVETo describe the early experiences with rFVIIa in the management of bleeding after cardiovascular surgery.

METHODSFrom May 2006 through December 2007, 16 patients received rFVIIa during or after surgery despite conventional medical therapy and transfusion of blood products. There were 15 male patients and 1 female patients, aged from 36 to 77 years old with a mean of 52 years old. The surgical procedures include aortic procedures for 8 cases, valve replacement for 6 cases, pulmonary thromboendarterectomy for 1 case and atrial septal defect repair for 1 case. The data of these patients were reviewed and the safety and efficacy of rFVIIa after cardiovascular surgery were evaluated.

RESULTSrFVIIa was administered as a first dose of 27.6 to 54.5 microg/kg with a mean of 40.2 microg/kg. Six patients achieved hemostasis after the first dose. Nine patients received a second administration within 30 min, with a cumulative dose of 59.3 to 90.9 microg/kg, a mean of 80.3 microg/kg. Eight patients achieved hemostasis and 1 patient went to exploration. One patient received four doses of rFVIIa with a cumulative dose of 203.4 microg/kg and the bleeding stopped. Mean amount of chest drain loss and the amount of red blood cell, fresh frozen plasma, cryoprecipitate, and platelet transfusions decreased significantly after rFVIIa administration. The total amount of chest drain losses, transfusions of red blood cell and cryoprecipitate within 12 h postoperatively was positively correlated with the time from the end of bypass to administration of rFVIIa. No thromboembolic complications and other adverse reactions were noted.

CONCLUSIONSThe use of rFVIIa is associated with reduced blood loss, rapid improvement of coagulation variables, and decreased need for blood products. rFVIIa is safe and efficacious in the management of refractory postcardiotomy bleeding.

Adult ; Aged ; Cardiovascular Surgical Procedures ; Coagulants ; administration & dosage ; therapeutic use ; Factor VIIa ; administration & dosage ; therapeutic use ; Female ; Humans ; Male ; Middle Aged ; Postoperative Hemorrhage ; drug therapy ; Recombinant Proteins ; administration & dosage ; therapeutic use ; Retrospective Studies

BACKGROUND: Recent studies and case reports have shown that recombinant factor VIIa (rFVIIa) treatment is effective for reversing coagulopathy and reducing blood transfusion requirements in trauma patients with life-threatening hemorrhage. The purpose of this study is to evaluate the effect of rFVIIa treatment on clinical outcomes and cost effectiveness in trauma patients. METHODS: Between January 2007 and December 2010, we reviewed the medical records of patients who were treated with rFVIIa (N=18) or without rFVIIa (N=36) for life-threatening hemorrhage due to multiple traumas at the Emergency Department of Pusan National University Hospital in Busan, Korea. We reviewed patient demographics, baseline characteristics, initial vital signs, laboratory test results, and number of units transfused, and then analyzed clinical outcomes and 24-hr and 30-day mortality rates. Thromboembolic events were monitored in all patients. Transfusion costs and hospital stay costs were also calculated. RESULTS: In the rFVIIa-treated group, laboratory test results and clinical outcomes improved, and the 24-hr mortality rate decreased compared to that in the untreated group; however, 30-day mortality rate did not differ between the groups. Thromboembolic events did not occur in both groups. Transfusion and hospital stay costs in the rFVIIa-treated group were cost effective; however, total treatment costs, including the cost of rFVIIa, were not cost effective. CONCLUSIONS: In our study, rFVIIa treatment was shown to be helpful as a supplementary drug to improve clinical outcomes and reduce the 24-hr mortality rate, transfusion and hospital stay costs, and transfusion requirements in trauma patients with life-threatening hemorrhage.
Adult ; Aged ; Aged, 80 and over ; Factor VIIa/*therapeutic use ; Hemoglobins/analysis ; Hemorrhage/complications/*drug therapy/mortality ; Humans ; Middle Aged ; Multiple Trauma/*complications ; Partial Thromboplastin Time ; Platelet Count ; Prothrombin Time ; Recombinant Proteins/therapeutic use ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo find a kind of quick and effective haemostasis to decrease the mortality of severe bleeding.

METHODS18 severe bleeding patients with different cause received recombinant activated factor VIIa (rFVIIa) were analyzed retrospectively.

RESULTSOf total 18 cases with severe bleeding, 13 cases cured, 3 cases were effective, 2 cases ineffective. The total clinical effective rate is 88.89%. After using rFVIIa, the PT, APTT and fibrinogen level of 6 DIC patients returned to normal within 12 hours; 13 patients whose the amount of bleeding can be evaluated stopped bleeding quickly. The fastest onset time was 10 min.

CONCLUSIONrFVIIa can stanch severe bleeding for a variety of reasons rapidly and effectively, including coagulopathy, thrombocytopenia, and obstetric hemorrhage. Application of rFVIIa may decrease mortality, when conventional treatment is not valid.

Adult ; Aged ; Aged, 80 and over ; Blood Coagulation Disorders ; drug therapy ; Factor VIIa ; therapeutic use ; Female ; Hemorrhage ; drug therapy ; Humans ; Male ; Middle Aged ; Recombinant Proteins ; therapeutic use ; Retrospective Studies ; Treatment Outcome ; Young Adult