deficit en facteur VII

Abstract

Thesis title: The factor VII deficiency. Author: BENYERGUED Hadjer, ACHEM Sarra, AGGOUN Yassine, BELAL Nadjem Eddine. Key words: Factor VII; bleeding disorders; Hemorrhagic syndrome; Deficiency. Objective: Clinical-biological confrontation. Introduction: Factor VII deficiency is a rare, usually congenital, bleeding disorder with autosomal recessive inheritance and is characterized by impaired or impaired factor VII coagulation activity. Materials and methods: Our work is a retrospective descriptive analysis, comprising 34 cases of factor VII deficiency, having been hospitalized in the hematology hospital of Tedjini Damerdji- Tlemcen, during the period 2003-2020. Results: • There are 17 men and 16 women. • 12 % of patients come from a consanguineous marriage, the familial character found in 17 patients. • 32 % of cases presented with hemorrhagic syndrome. • All our patients have a low TP and a normal TCA with a factor VII level between 12% and 66%. Discussion: This deficiency is responsible for a hemorrhagic syndrome of varying intensity and miscorrelated most of the time to residual activity levels of factor VII. Currently, the treatment is of the substitutive type but the indications remain difficult to lay down in the absence of consensus particularly on the haemostatic rate of factor VII that should be reached. Conclusion: The aim of this work is to report several clinical cases and to make a literature review on the clinical, biological, genetic and therapeutic distinctive features of this rare disease.