Perioperative Hemostatic Management of a Pediatric Patient with Glanzmann Thrombasthenia Undergoing Osteoplastic Craniotomy and Hematoma Removal: A Case Report

Glanzmann thrombasthenia is an uncommon hereditary disease that involves an abnormal platelet function leading to complicated hemostatic problems. In situations of anticipated hemorrhage, irradiated apheresed platelets are the first line of treatment. In addition, a combination of recombinant factor VIIa and an antifibrinolytic agent such as tranexamic acid can be utilized to minimize bleeding. Here we are present stable management of a pediatric patient with Glanzmann thrombasthenia admitted for traumatic epidural hematoma removal. Due to the condition of the operation site, some blood loss was unavoidable. However, hemostasis was successfully controlled, and the patient was discharged without additional complications.

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Successful Use of Recombinant Factor VIIa (NovoSeven) During Cardiac Surgery in a Pediatric Patient with Glanzmann Thrombasthenia

Pediatric Cardiology ◽

10.1007/s00246-004-0919-7 ◽

2005 ◽

Vol 26 (6) ◽

pp. 843-845 ◽

Cited By ~ 26

Author(s):

B.T. Yilmaz ◽

B. Alioglu ◽

E. Ozyurek ◽

H.T. Akay ◽

S. Mercan ◽

...

Keyword(s):

Cardiac Surgery ◽

Pediatric Patient ◽

Recombinant Factor Viia ◽

Factor Viia ◽

Glanzmann Thrombasthenia

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Faculty Opinions recommendation of Changing from aprotinin to tranexamic acid results in increased use of blood products and recombinant factor VIIa for aortic surgery requiring hypothermic arrest.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.7865956.8351061 ◽

2011 ◽

Author(s):

Ronald Pearl ◽

Jim Wong

Keyword(s):

Tranexamic Acid ◽

Recombinant Factor Viia ◽

Factor Viia ◽

Aortic Surgery ◽

Blood Products ◽

Hypothermic Arrest

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Improved hemostasis with superactive analogs of factor VIIa in a mouse model of hemophilia A

Blood ◽

10.1182/blood-2003-05-1369 ◽

2003 ◽

Vol 102 (10) ◽

pp. 3615-3620 ◽

Cited By ~ 82

Author(s):

Mikael Tranholm ◽

Kim Kristensen ◽

Annemarie T. Kristensen ◽

Charles Pyke ◽

Rasmus Røjkjær ◽

...

Keyword(s):

Blood Loss ◽

Bleeding Time ◽

Hemophilia A ◽

Recombinant Factor Viia ◽

Factor Viia ◽

Intrinsic Activity ◽

Hemostatic Effect ◽

Therapeutic Doses ◽

Hemostatic Effects

AbstractIt is currently debated whether the mechanism of action of therapeutic doses of recombinant factor VIIa (rFVIIa, Novo-Seven) relies on the tissue factor (TF)-independent activity of the enzyme. The present study was conducted to investigate the in vivo hemostatic effects of rFVIIa and 3 analogs thereof with superior intrinsic activity (FVIIaIIa, K337A-FVIIaIia, and M298Q-FVIIa) in mice with antibody-induced hemophilia A. A highly significant dose response was observed for the bleeding time and blood loss for each of the rFVIIa variants. The bleeding time and blood loss were normalized after administration of 10 mg/kg rFVIIa, 3 mg/kg K337A-FVIIaIia, and 3 mg/kg M298Q-FVIIa, indicating a potency of these FVIIa analogs 3-4 times above that of rFVIIa in FVIII-depleted mice. The different in vivo potencies of the various forms of FVIIa could not be explained by the pharmacokinetics. Histopathological evaluation of kidneys revealed no signs of treatment-related pathological changes even after treatment with the superactive variants. The fact that FVIIa analogs with enhanced intrinsic activity are more efficacious in the murine hemophilia A model strongly suggests that the TF-independent procoagulant activity of FVIIa contributes to its clinical hemostatic effect. (Blood. 2003; 102:3615-3620)

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