Potential predictors of bleeding risk in inherited factorVII deficiency

Abstract

SummaryDue to the wide molecular and clinical heterogeneities of inherited factor VII (FVII) deficiency, consensus guidelines for management of this coagulation disorder are not currently well established. Therefore, potential clinical, plasmatic or genetic criteria, that could be predictive for bleeding tendency in this condition, have been evaluated. Genotypic criteria including FVII genotypes and thrombophilic mutations are of particular interest to better understand some of the variations observed in bleeding phenotypes but they are still poorly informative for the management of surgery in FVII-deficient patients. Up to now, no plasma parameters have been found to be reliable predictors of bleeding risk. Nevertheless, tissue factor and platelet pathways remain to be explored. Finally, clinical history appears to be the best predictor of bleeding risk after haemostatic challenges in inherited FVII deficiencies. Furthermore, the absence of history of bleeding or mild bleeding phenotypes including menorrhagia, bruises and epistaxis (not inducing iron deficiency anaemia or requiring blood substitutive treatment) could enable minor surgery to be performed in FVII-deficient patients without blood replacement therapy.