title: How we manage patients with hereditary haemochromatosis.
abstract: A number of disorders cause iron overload: some are of genetic origin, such as hereditary haemochromatosis, while others are acquired, for instance due to repeated transfusions. This article reviews the treatment options for hereditary haemochromatosis, with special attention to the use of erythrocytapheresis. In general, therapy is based on the removal of excess body iron, for which ferritin levels are used to monitor the effectiveness of treatment. For many decades phlebotomy has been widely accepted as the standard treatment. Recent publications suggest that erythrocytapheresis, as a more individualized treatment, can provide a good balance between effectiveness, tolerability and costs. Other treatments like oral chelators and proton pomp inhibitors, which are used in selected patients, create the possibility to further individualize treatment of hereditary haemochromatosis. In the future, hepcidin-targeted therapy could provide a more fundamental approach to treatment.
authors.affiliation: Division of Gastroenterology & Hepatology, Department of Internal Medicine, Maastricht University Medical Centre, Maastricht, the Netherlands.|Department of Transfusion Medicine, Sanquin Blood Supply, Amsterdam, the Netherlands.
authors.forename: Eva
authors.initials: E
authors.lastname: Rombout-Sestrienkova
authors.affiliation: Department of Transfusion Medicine, Sanquin Blood Supply, Amsterdam, the Netherlands.
authors.forename: Marian G J
authors.initials: MG
authors.lastname: van Kraaij
authors.affiliation: Division of Gastroenterology & Hepatology, Department of Internal Medicine, Maastricht University Medical Centre, Maastricht, the Netherlands.
authors.forename: Ger H
authors.initials: GH
authors.lastname: Koek
mesh_terms: D016238:Cytapheresis; D019468:Disease Management; D004912:Erythrocytes / Q000166:cytology; D006432:Hemochromatosis / Q000628:therapy*; D064451:Hepcidins / Q000037:antagonists & inhibitors; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D054328:Proton Pump Inhibitors / Q000627:therapeutic use
chemical_list: D064451:Hepcidins; D007502:Iron Chelating Agents; D054328:Proton Pump Inhibitors
keywords: erythrocytapheresis; hereditary haemochromatosis; phlebotomy; treatment