LAUSR

Hereditary Hemochromatosis is a condition caused by defects in iron-sensing genes leading to parenchymal iron loading. If diagnosed early and treated appropriately, many of the complications, including liver fibrosis, cirrhosis and liver cancer, arthritis, cardiomyopathy and diabetes, were thought to be avoided. As iron reduction by venesection became the mainstay of HH treatment before the introduction of evidence-based medicine, its efficacy has never been the subject of high-level clinical research. Here we provide a systematic review of iron reduction in HH, including 24 studies and 6000 patients. While strong deductions are prohibited by an absence of robust clinical trial data, the purported benefits of venesection are reviewed and we report an improvement in fatigue, liver function tests and fibrosis, and overall survival. In conclusion, these findings, coupled with the absence of an alternative, low-cost, effective and tolerable therapy, suggests venesection will remain the mainstay of treatment in HH for decades to come.