INHIBITOR DEVELOPMENT IN CONGENITAL FACTOR VII DEFICIENCY – CASE PRESENTATION
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Abstract
Congenital factor VII defi ciency is a rare disease. Inhibitor development in congenital factor VII defi ciency is an extreme rare phenomen, as the main challenge of replacement therapy by a high rate of life-threatening bleeding, that renders replacemrnt therapy less eff ective. Due to the important of the issue, we would like to present this case-report, It was a female 11 months with congenital factor VII defi ciency experiencing recurrent epistaxis, hematoma, intracranien
hemorrhages from second month of life, and successful treatment by double dose of recombinant activated VII. Some conclusions are that, inhibitor detection in congenital factor VII defi ciency with severe recurrent hemorrhage or less successful of replacement therapy is always needed; and high dose of rFVIIa in treatment of congenital factor VII defi ciency can successfully stop bleeding.
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Keywords
Factor VII defi ciency, Factor VII Inhibitor, Replacement therapy
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