Hereditary FACTOR VII Deficiency: About Two Cases
Keywords:Coagulation factor VII, Hereditary deficiency, hemorrhagic manifestations
Coagulation factor VII or proconvertin is a vitamin K dependent glycoprotein synthesized by the liver. This factor initiates the coagulation cascade by binding to tissue factor after a vascular breach. A qualitative or quantitative deficiency in this factor can cause hemorrhagic manifestations of varying severity. Hereditary factor VII deficiency is a rare disease. The clinical symptomatology is very variable and not correlated to the deficiency in this factor. It can be fatal with a severe hemorrhagic syndrome. An isolated prolongation of the prothrombin time is the first assessment that points to this diagnosis and a low level of plasma factor VII activity confirms it. The Treatment is very complex, hence the importance of collaboration between clinicians and biologists.
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