BOF: General (Iron)

Which ONE of the following five statements regarding venesection for the treatment of hereditary haemochromatosis is MOST accurate:

a) Initial phlebotomy strategy should be to remove of 1-2 units of blood per month until ferritin level is <50ng/ml.

b) Iron deficiency anaemia is frequently a consequence of over-aggressive phlebotomy-based therapy

c) Once end-organ damage such as cirrhosis and hypogonadism is established, its progression cannot be reversed by treatment.

d) Therapeutic phlebotomy in combination with iron chelation therapy offers the most effective means of preventing end-organ damage.

e) Treatment can be deferred until ferritin level is >2000ng/ml.

Answer:

c) Once end-organ damage such as cirrhosis and hypogonadism is established, its progression cannot be reversed by treatment.

Explanation

In treating hereditary haemochromatosis, early therapy can prevent end organ damage but will only slow the progression of established damage, not reverse it. Therapeutic phlebotomy may be deferred but should commence once ferritin reaches 1000ng/ml if diagnosis is made early, initially at a rate of 1-2 units every 1-2 weeks as tolerated, with a target ferritin of <50ng/ml and transferrin saturation of <50%. Maintenance therapy should then be commenced. There is no place for chelation therapy.

Reference:

Diagnosis and management of hemochromatosis: 2011 practice guideline by the American Association for the Study of Liver Diseases. Hepatology 2011; 54:328.