Hemochromatosis due to the HFE mutation C282Y causes a progressive iron overload. The mortality occurs after 50 years. Repeated bleeding is an effective treatment with marginal side effects. The rationale for screening, debated for 60 years, depends on the penetrance and the effectiveness of treatment relatively to economic and social issues. This work provides evidence from cohort LOGIFER, 25 years old at the CHU of Rennes, composed of 1835 homozygous: consultants from CHU, from targeted surveys, referred to the NRC or related. The reasons for recruitment, gender and age have changed. 2/3 of patients are probants, half of them coming from screening, providing better representativity. The sex ratio was 1.2. They are characterized by skin pigmentation and liver disease. Joint involvement are nonspecific. Traditional measures of overload are unequivocal , highly heterogeneous among individuals and increases until at least 60 years. Women store iron slowly until menopause. Alcohol and metabolic syndrome worse overload. Alcohol accelerates the clinical discover. Metabolic syndrome creates a moderate overload, associated arthropathies involve other joints. Approximately 20% of clinical signs at diagnosis contribute to the penetrance. The Iron removal index is based on the amount of iron removed to maintain iron status . It has two usages probably valid for other hemochromatosis. Measuring the rate of absorption of iron for a specific patient, supplementing the phenotype and giving a dynamic view of the risk of overload unlike conventional criteria. Providing a framework for controlling the bleeding pattern. The processing rate is customizable and predictable at diagnosis (age, sex, fibrosis, ferritin target, weight). Con,sequently, changes in iron status during maintaining treatment involves complementary explorations. Genotyped patients monitored for 8.5 years during treatment have a mortality rate of 5%. At 54 years, there is no excess mortality after adjustment for sex, place and year of birth. There is a significant hepatic mortality especially primary liver cancers (ICM=18). They die within 5 years of diagnosis. It is offset by a subsequent under mortality from heart diseases or other cancers. Life expectancy down for patients initially enrolled in part due to delayed diagnosis and less effective adjuvant treatments. The age at death is 64 to 68 years. The effect of phelbotomies is not quantifiable but advocates for early treatment. The Guyader's predictor of cirrhosis adapts to the prediction of fibrosis (similar Youden index). Discriminant analysis predicts METAVIR stages with the benefit of a reliability check. These equations are the best predictors among candidates proposed by the literature. Development goes through the implementation of longitudinal studies between 65 and 80 years and the study of non-lethal forms.
