Recombinant activated factor VII and epsilon aminocaproic acid treatment of a patient with Glanzmann's thrombasthenia for nasal polipectomy

2007 ◽  
Vol 21 (1) ◽  
pp. 106-107 ◽  
Author(s):  
Berrin Gunaydin ◽  
Zerrin Ozkose ◽  
Seyda Pezek
Keyword(s):  
Acid Treatment ◽  
Aminocaproic Acid ◽  
Factor Vii ◽  
Recombinant Activated Factor Vii ◽  
Glanzmann’S Thrombasthenia ◽  
Activated Factor Vii ◽  
Glanzmann's Thrombasthenia ◽  
Epsilon Aminocaproic Acid

scholarly journals The Use of Recombinant Activated Factor VII in Patients with Glanzmann's Thrombasthenia

2020 ◽  
Author(s):  
Man-Chiu Poon
Keyword(s):  
Unmet Need ◽  
Factor Vii ◽  
European Medicines Agency ◽  
Recombinant Activated Factor Vii ◽  
Glanzmann’S Thrombasthenia ◽  
Activated Factor Vii ◽  
Safety Surveillance ◽  
Glanzmann's Thrombasthenia ◽  
Bleeding Episodes ◽  
Surveillance Database

AbstractPlatelet transfusion is the standard treatment to control or prevent bleeding in patients with Glanzmann's thrombasthenia (GT), but platelets are often unavailable. Recombinant activated factor VII (rFVIIa) is an effective alternative to platelets in patients with GT with past/present refractoriness to platelet transfusions and antibodies to platelets. However, there is an unmet need for an alternative to platelets in patients without antibodies. This report summarizes evidence of efficacy and safety of rFVIIa in patients with GT without refractoriness or antibodies to platelets from three different sources: the Glanzmann's Thrombasthenia Registry (GTR), published literature (January 01, 1999 to December 01, 2017), and the Novo Nordisk safety surveillance database. In the GTR, 133 patients received rFVIIa for the treatment of 333 bleeding episodes and prevention of bleeding in 157 surgical procedures. Overall efficacy rates were 79 and 88%, respectively, in patients treated for bleeding episodes or for the prevention of bleeding during surgery; effectiveness was generally similar across refractoriness/antibody status categories. Median dose per infusion of rFVIIa was close to that recommended for patients with GT (90 µg/kg). Data from 14 published case reports also demonstrated that rFVIIa is effective with an acceptable safety profile in patients with GT without antibodies to platelets. Analysis of adverse events reported in GTR and in Novo Nordisk safety surveillance database did not raise any new safety concerns. These data supported the label extension of rFVIIa to include cases where platelets are not readily available, which was approved by the European Medicines Agency in December 2018.

scholarly journals The use of recombinant activated factor VII in the treatment of gastrointestinal bleeding following acetylsalicylic acid therapy in a surgical patient

2008 ◽  
Vol 136 (Suppl. 3) ◽  
pp. 240-245 ◽  
Author(s):  
Dragica Vucelic ◽  
Predrag Sabljak ◽  
Predrag Pesko ◽  
Dejan Stojakov ◽  
Keramatollah Ebrahimi ◽  
...  
Keyword(s):  
Gastrointestinal Bleeding ◽  
Acetylsalicylic Acid ◽  
Acid Treatment ◽  
Haemorrhagic Shock ◽  
Factor Vii ◽  
Surgical Patient ◽  
Recombinant Activated Factor Vii ◽  
Acid Therapy ◽  
Activated Factor Vii ◽  
Massive Gastrointestinal Bleeding

INTRODUCTION. Gastrointestinal bleeding is the most important complication associated with acetylsalicylic acid therapy. Patients with preexisting haemostatic disorders are at the higher risk and may experience life-threatening hemorrhagic syndrome. Platelet transfusions and desmopressin administration commonly successfully arrest bleeding. However, in clinical situations with profound bleeding and haemorrhagic shock, these therapeutic approaches may fail. CASE OUTLINE. We report a 24-year old female patient with previously undetected acquired platelet dysfunction, who underwent reconstructive surgical intervention. On the 20th postoperative day, acetylsalicylic acid was introduced due to reactive thrombocytosis (platelet count 1480x109/L) with daily dose of 100 mg tablets. On the 12th day of the acetylsalicylic acid treatment, massive gastrointestinal bleeding with haemorrhagic shock suddenly occurred. Attempts to control massive haemorrhage by resuscitation, blood products and haemostatics (desmopressin, tranexamic acid) failed. Two bolus doses of recombinant activated factor VII (rFVIIa) (100 ?g/kg and 60 ?g/kg respectively) in 90 minutes interval were given. Bleeding was successfully controlled with no requirements for further haemoproducts and haemostatic remedies treatment. CONCLUSION. This case demonstrates that the use of rFVIIa may be a specific treatment option in patients suffering from severe gastrointestinal bleeding associated with acetylsalicylic acid treatment.

Alloimmunization in Congenital Deficiencies of Platelet Surface Glycoproteins: Focus on Glanzmann's Thrombasthenia and Bernard–Soulier's Syndrome

2018 ◽  
Vol 44 (06) ◽  
pp. 604-614 ◽  
Author(s):  
Roseline d'Oiron ◽  
Man-Chiu Poon
Keyword(s):  
Platelet Transfusion ◽  
Clinical Laboratory ◽  
Human Leukocyte ◽  
Factor Vii ◽  
Platelet Glycoprotein ◽  
Recombinant Activated Factor Vii ◽  
Platelet Surface ◽  
Glanzmann’S Thrombasthenia ◽  
Platelet Antibodies ◽  
Glanzmann's Thrombasthenia

AbstractGlanzmann's thrombasthenia (GT) and Bernard–Soulier's syndrome (BSS) are well-understood congenital bleeding disorders, showing defect/deficiency of platelet glycoprotein (GP) IIb/IIIa (integrin αIIbβ3) and GPIb-IX-V complexes respectively, with relevant clinical, laboratory, biochemical, and genetic features. Following platelet transfusion, affected patients may develop antiplatelet antibodies (to human leukocyte antigen [HLA], and/or αIIbβ3 in GT or GPIb-IX in BSS), which may render future platelet transfusion ineffective. Anti-αIIbβ3 and anti-GPIb-IX may also cross the placenta during pregnancy to cause thrombocytopenia and bleeding in the fetus/neonate. This review will focus particularly on the better studied GT to illustrate the natural history and complications of platelet alloimmunization. BSS will be more briefly discussed. Platelet transfusion, if unavoidable, should be given judiciously with good indications. Patients following platelet transfusion, and women during and after pregnancy, should be monitored for the development of platelet antibodies. There is now a collection of data suggesting the safety and effectiveness of recombinant activated factor VII in the management of affected patients with platelet antibodies.

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