scholarly journals Ten-year experience of recombinant activated factor VII use in surgical patients with congenital haemophilia with inhibitors or acquired haemophilia in Japan

Haemophilia ◽  
2014 ◽  
Vol 21 (3) ◽  
pp. 374-379 ◽  
Author(s):  
H. Takedani ◽  
M. Shima ◽  
Y. Horikoshi ◽  
T. Koyama ◽  
K. Fukutake ◽  
...  
Keyword(s):  
Surgical Patients ◽  
Factor Vii ◽  
Recombinant Activated Factor Vii ◽  
Acquired Haemophilia ◽  
Activated Factor Vii

The use of recombinant activated factor VII in patients with acquired haemophilia

Blood Reviews ◽  
2015 ◽  
Vol 29 ◽  
pp. S19-S25 ◽  
Author(s):  
Andreas Tiede ◽  
Kagehiro Amano ◽  
Alice Ma ◽  
Per Arkhammar ◽  
Soraya Benchikh el Fegoun ◽  
...  
Keyword(s):  
Factor Vii ◽  
Recombinant Activated Factor Vii ◽  
Acquired Haemophilia ◽  
Activated Factor Vii

scholarly journals Acquired Haemophilia A. Which is the best therapeutic choice in older adults? Single center study of 4 cases

Reumatismo ◽  
2019 ◽  
Vol 71 (1) ◽  
pp. 37-41 ◽  
Author(s):  
E. Mauro ◽  
E. Garlatti Costa ◽  
A. Zanier ◽  
M. Maset ◽  
A. Ermacora ◽  
...  
Keyword(s):  
Coagulation Factor ◽  
Factor Vii ◽  
Haemophilia A ◽  
Recombinant Activated Factor Vii ◽  
Acquired Haemophilia ◽  
Activated Factor Vii ◽  
Activated Prothrombin Complex Concentrate ◽  
Therapeutic Choice ◽  
Single Center Study ◽  
Thrombotic Complications

Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies directed against coagulation factor VIII. The treatment is based on recombinant activated factor VII and activated prothrombin complex concentrate. However, mainly in older patients, severe thrombotic complications have been reported. Here we report the different therapeutic approaches in 4 cases of elderly patients with AHA and co-morbidities.

scholarly journals The Effects of Acidosis and Hypothermia on Blood Transfusion Requirements following Factor VII Administration

2007 ◽  
Vol 35 (4) ◽  
pp. 494-497 ◽  
Author(s):  
K. Hall ◽  
P. Forrest ◽  
C. Sawyer
Keyword(s):  
Blood Transfusion ◽  
Rescue Therapy ◽  
Surgical Patients ◽  
Factor Vii ◽  
Red Cell ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Rfviia Administration ◽  
Transfusion Requirements ◽  
Red Cell Transfusion

While there is laboratory evidence that the activity of recombinant activated factor VII (rFVIIa) is reduced by the presence of acidosis and hypothermia, there is limited clinical data to support this observation. Recombinant FVIIa may be used as rescue therapy in surgical patients who have bleeding that is refractory to conventional therapy. However, these patients are also frequently acidotic and hypothermic at the time the drug is administered. In this retrospective study, the records of 38 adult surgical patients who received rFVIIa intraoperatively or within six hours postoperatively were reviewed. The requirements for red cell transfusion in the two hours following the administration of rFVIIa and the need for repeated doses of rFVIIa were recorded. The relationship between red cell transfusion and pH and temperature of the patient at the time of rFVIIa administration was assessed by multiple regression analysis. The major finding was an inverse relationship between the degree of acidosis at the time of rFVIIa administration and the requirement for either subsequent blood transfusion or repeat dosing of rFVIIa (P=0.003 and P <0.001 respectively). For patients with apH <7.2 vs. pH >7.2, the odds ratio for receiving two or more packs of red blood cells within two hours of rFVIIa administration was 15:1. This effect was not observed for hypothermia. The implication of this study is that rFVIIa may be less effective when administered to severely acidotic patients. Further studies are required to examine whether this is related to the acidosis directly, or is secondary to other intraoperative variables affecting acidosis. The clinical utility of rFVIIa in acidotic patients also requires further investigation.

scholarly journals Acquired haemophilia: a case report and a clinical review

2013 ◽  
pp. 47-49
Author(s):  
Lucia Todaro ◽  
Giuseppe Leone ◽  
Mauro Girotto ◽  
Pierdomenico Bertello
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Medical Emergency ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Laboratory Findings ◽  
Recombinant Activated Factor Vii ◽  
Acquired Haemophilia ◽  
Activated Factor Vii ◽  
Prolonged Aptt

BACKGROUND Acquired haemophilia presents later in life and is due to acquired inhibitors to factor VIII. The disorder is very rare (about one case per million population per year) but causes significant morbidity and mortality. CASE REPORT We report the case of a 57 year-old obese man who was admitted to our Day Hospital for anemia. At admission we observed diffuse ecchymoses and a large subcutaneous hematoma in the inferior right hemithorax. Laboratory findings showed severe anemia, a prolonged aPTT with a normal PT and platelet count. The titer of anti-factor VIII inhibitor in Bethesda units resulted very high and confirmed the diagnosis of acquired haemophilia. We used recombinant activated factor VII (rFVIIa) to control active bleeding and prednisone, cyclophosphamide and, lastly, the anti- CD 20 monoclonal antibody (rituximab) to eliminate the factor VIII inhibitors. Unfortunately, on fourth week, the patient suffered an extensive hematoma of the abdominal wall and died. CONCLUSIONS We underline the importance of not overlooking a prolonged aPTT because the bleedings associated with acquired haemophilia can be life-threatening and constitute a medical emergency.

scholarly journals Adaptation of recombinant activated factor VII in the treatment of acquired haemophilia A: Results from a prospective study (ACQUI-7) in France

2021 ◽  
Vol 2 ◽  
pp. 100021
Author(s):  
Benoît Guillet ◽  
Achille Aouba ◽  
Annie Borel-Derlon ◽  
Jeanne Yvonne Borg ◽  
Jean-François Schved ◽  
...  
Keyword(s):  
Prospective Study ◽  
Factor Vii ◽  
Haemophilia A ◽  
Recombinant Activated Factor Vii ◽  
Acquired Haemophilia ◽  
Activated Factor Vii ◽  
A Prospective Study
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