LONG-TERM RESULTS OF VENESECTION THERAPY IN IDIOPATHIC HEMOCHROMATOSIS

  • 1 January 1976
    • journal article
    • research article
    • Vol. 45  (180) , 611-623
Abstract
Observations on the clinical effects of venesection therapy in 85 treated, as compared with 26 untreated, patients with idiopathic hemochromatosis showed decreased pigmentation and hepatomegaly together with a return to normal tests of liver function in half the patients who had abnormal tests at presentation. Control improved in 28% of those patients with diabetes mellitus, although some patients developed it during the period of observation, despite venesection. Portal hypertension, testicular atrophy and arthropathy were not improved. In only 12 patients was there sufficient reaccumulation of Fe, after the initial course of venesection, to merit further treatment. Rates of Fe accumulation in these patients varied between 1.4 mg-4.8 mg/day and chelatable Fe levels were noted to be inappropriately high in relation to body Fe stores during the early stages of the reaccumulation period. Life table data showed that the percentage survival 5 and 10 yr after diagnosis was 66 and 32%, respectively, for the treated patients, and 18 and 6%, respectively, for the untreated patients, both statistically highly significant differences (P < 0.01). Possible clinical differences such as age of presentation, the presence of diabetes mellitus, cirrhosis, clinical hepatic failure and hepatoma between the treated and untreated groups that might otherwise have weighted survival in favor of the treated group were corrected by the use of covariant analysis. This gave mean log survival values of 4.15 and 2.88 for the treated and untreated patients, respectively, equivalent to 63.4 mo. and 17.8 mo., a highly significant difference (P < 0.01). Ten patients, all of whom had cirrhosis at the time of diagnosis, died of malignant hepatoma between 3-15 yr after completing venesection therapy. There was also a high rate of death from neoplasms in a variety of other sites, 22% in the venesected group, strikingly higher than the rate predicted for a similarly aged population using national cancer mortality rates.

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