A Preoperative Evaluation Revealing a Rare Coagulation Factor Deficiency

Abstract

Constitutional factor II deficiency is a rare coagulation disorder. The autosomal recessive form has an estimated prevalence of 1-2 million. Clinical symptoms depend on the rate of factor II deficiency. We report a fortuitous discovery of an isolated factor II deficiency in a 9-year-old girl of 1st degree consanguineous parents, following a preoperative assessment of drainage of her abundant pericardial effusion, the biological assessment showed a factor II rate < 1%, a prothrombin rate at 3% and a TCA > 120 sec, the dosage of the other coagulation factors are without anomalies. Through this observation we recall the rarity of this deficit and thought about it in the face of unusual bleeding in newborns of consanguineous parents, as well as the necessity of collaboration with international registries to establish codified diagnostic and therapeutic guidelines. Keywords: Factor II; Congenital Hemorrhagic Diseases; Rare Coagulation Factor Deficiency Abbreviations: FFP: Fresh Frozen Plasma