Long Term Results of Venesection Therapy in Idiopathic Haemochromatosis

Observations on the clinical effects of venesection therapyin 85 treated, as compared with 26 untreated, patients withidiopathic haemochromatosis showed decreased pigmentation andhepatomegaly together with a return to normal tests of liverfuntion in half the patients who had abnormal tests at presentation.Control improved in 28 per cent of those patients with diabetesmellitus, although some patients developed it during the periodof observation, despite venesection. Portal hypertension, testicularatrophy and arthropathy were not improved. In only 12 patientswas there sufficient reaccumulation of iron after the initialcourse of venesection to merit further treatment. Rates of ironaccumulation in these patients varied between 1·4 mgand 4·8 mg per day and chelatable iron levels were notedto be inappropriately high in relation to body iron stores duringthe early stages of the reaccumulation period. Life table datashows that the percentage survival five and ten years afterdiagnosis was 66 and 32 per cent respectively for the treatedpatients, and 18 and 6 per cent respectively for the untreatedpatients, both statistically highly significant differences(p < 0·01). Possible clinical differences such asage of presentation, the presence of diabetes mellitus, cirrhosis,clinical hepatic failure and hepatoma between the treated anduntreated groups that might otherwise have weighted survivalin favour of the treated group were corrected by the use ofcovariant analysis. This gave mean log survival values of 4·15and 2·88 for the treated and untreated patients respectively,equivalent to 63·4 months and 17·8 months, a highlysignificant difference (p < 0·01). Ten patients, allof whom had cirrhosis at the time of diagnosis, died of malignanthepatoma between three and 15 years after completing venesectiontherapy. There was also a high rate of death from neoplasmsin a variety of other sites—22 per cent in the venesectedgroup, strikingly higher than that rate predicted for a similarlyaged population using national cancer mortality rates.