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Successful neurosurgery of a malignant glioneuronal tumor (MGT) under recombinant activated factor VII (rFVIIa) cover in a severe hemophilia a patient with a high titer inhibitor (SHHI)

Abstract number: PP-WE-592

Aouba¹ A., Dezamis² E., Rothschild¹ C., Torchet¹ M.

¹¹Hematology and Hemophilia Center, Necker Hospital ²²Neurosurgery, Sainte Anne Hospital, Paris, France

How-to-cite Aouba A, Dezamis E, Rothschild C, Torchet M. Successful neurosurgery of a malignant glioneuronal tumor (MGT) under recombinant activated factor VII (rFVIIa) cover in a severe hemophilia a patient with a high titer inhibitor (SHHI). Journal of Thrombosis and Haemostasis 2009; Volume 7, Supplement 2: Abstract PP-WE-592

The development of inhibitors following FVIII replacement therapy is a severe complication in hemophilia. Whereas significant experience, notably in orthopedic surgery, is now obtained with the use of bypassing agents in SHHI, new surgical challenges might occur due to patients’ increasing life expectancy.

A 56 year-old SHHI was diagnosed for probable right temporo-parietal MGT on cerebral MRI (4 × 3 cm cerebromeningeal tumor with perilesional edema and transfalcial herniation) requiring total resection. Previously, the patient has experienced several hemarthroses which were treated with aPCC (Feiba^¯). At admission the inhibitor titer was 16 BU; surgery was programmed within the following 3 weeks because of pyramidal signs. To allow the use of high-dose FVIII during surgery, Rituximab was initiated to lower the inhibitor titer < 5 BU, but unsuccessfully. Therefore rFVIIa (NovoSeven^¯) was chosen as the hemostatic agent: bolus of 270 μg kg^–1 2 h^-1during the first 24 h, 180 μg kg^-1 3 h^-1, 4 h^-1, 6 h^-1 respectively at days 2 and 3, from days 4–10 and finally from days 11–15. Tranexamic acid was associated. Per- and post-operative courses were uneventful, the surgical procedure being assessed at optimal haemostatic condition without any unusual hemorrhage on MRI controls, DIC criteria or thromboembolic event.

Intensive rFVIIa therapy has shown to be safe and effective in this first ever reported neurosurgery in SHHI of a malignant tumor with a high bleeding risk. The use of lower doses of rFVIIa might have been possible; however, in the absence of a thrombin generation test, the potentially life-threatening complications of this procedure required maximum hemostasis with high rFVIIa doses.

Disclosure of interest: none declared.

To cite this abstract use the following format:

Journal of Thrombosis and Haemostasis 2007; Volume 5, Supplement 2: abstract number

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Session name:	ISTH2009
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