title: [Survival and development of neoplasms in 56 patients with idiopathic hemochromatosis].
abstract: Fifty-six patients with idiopathic hemochromatosis (50 men and 6 women, mean age 49.7 +/- SD 11.1 and 60.8 +/- SD 10.3 years respectively) were studied and followed up for a median period of 44 months (range 3-168). At basal liver biopsy 44 patients had cirrhosis and 12 fibrosis. Iron depletion was achieved in 39 of the 46 cases who underwent iron removal by erythrocytapheresis. Eighteen patients died, 11 from malignancy and 7 from other causes. A total of 14 malignant neoplasms were observed (6 hepatocellular and 8 extrahepatic), of which 6 (5 hepatocellular and 1 pancreatic) were already evident at enrollment. Cumulative survival rates at 3, 5 and 8 years were 75.4%, 64.2% and 54.4% respectively, significantly lower than those in the general population. Probabilities of developing cancer in the 50 patients without cancer at diagnosis were 5.5%, 14.9% and 44.4% at 3, 5 and 8 years respectively.
authors.affiliation: Cattedra di Patologia Medica III, Istituto di Scienze Mediche, Università degli Studi di Milano.
authors.forename: M
authors.initials: M
authors.lastname: Fraquelli
authors.forename: C
authors.initials: C
authors.lastname: Mandelli
authors.forename: L
authors.initials: L
authors.lastname: Cesarini
authors.forename: D
authors.initials: D
authors.lastname: Barisani
authors.forename: P A
authors.initials: PA
authors.lastname: Bianchi
authors.forename: D
authors.initials: D
authors.lastname: Conte
mesh_terms: D006528:Carcinoma, Hepatocellular / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006432:Hemochromatosis / Q000150:complications* / Q000175:diagnosis / Q000401:mortality*; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology; D008113:Liver Neoplasms / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000209:etiology*; D011336:Probability; D013997:Time Factors
vernacular_title: Sopravvienza e sviluppo di neoplasie in 56 pazienti con emocromatosi idiopatica.