Treatment of hemochromatosis
| Optimization of: . | Treatment and prevention of: . | Treatment of: . | |
|---|---|---|---|
| LIFESTYLE . | IRON OVERLOAD . | COMPLICATIONS . | |
| • MINIMIZE OR AVOID ALCOHOL • HEALTHY DIET • REGULAR PHYSICAL ACTIVITY, MAINTAIN IDEAL WEIGHT • AVOID RAW OR UNDERCOOKED SEAFOOD AND WOUND CONTACT WITH SEAWATER | • PHLEBOTOMY | Induction phase 350-500 mL (according to sex/weight) every 1-2 weeks. Check Hb before each phlebotomy (discontinue/delay if <11-12 g/dL) and ferritin every 4 phlebotomies (more frequently when ferritin <200 mg/L). Goal: ferritin ~50 mg/L. Maintenance phase 2-4 phlebotomies per year to keep ferritin within the goal of ~50-100 mg/L. Patients may be usefully enrolled as regular blood donors. Typically well tolerated. Problematic if hemodynamic instability (advanced liver or heart failure), poor venous access, needle phobia, living away from health care facilities. | • LIVER AND HEART FAILURE Erythrocytapheresis preferable over phlebotomy (isovolemic procedure). Deferoxamine in heart failure during the first phases. • HORMONE REPLACEMENT THERAPY (Eg, insulin, androgens) • ARTHROPATHY (May not respond to phlebotomy). Analgesia, physiotherapy, joint replacement • OSTEOPOROSIS Biphosphonates, vitamin D |
| • ERYTHROCYTAPHERESIS | More rapid iron depletion. Cost issues (equipment, personnel). It can preserve blood volume. | ||
| • IRON-CHELATING AGENTSa | Deferoxamine (parenteral), or deferasirox and deferiprone (oral). Use limited to patients with severe life-threatening IO (alone or in combination) or intolerant to phlebotomy. A careful evaluation of the risk-benefit ratio is needed, and they should be prescribed by clinicians with expertise in IO disorders. | ||
| • HEPCIDIN MIMETICS | Rusfertide (weekly SC injections) promising for maintenance phase (needs confirmation). | ||
| Optimization of: . | Treatment and prevention of: . | Treatment of: . | |
|---|---|---|---|
| LIFESTYLE . | IRON OVERLOAD . | COMPLICATIONS . | |
| • MINIMIZE OR AVOID ALCOHOL • HEALTHY DIET • REGULAR PHYSICAL ACTIVITY, MAINTAIN IDEAL WEIGHT • AVOID RAW OR UNDERCOOKED SEAFOOD AND WOUND CONTACT WITH SEAWATER | • PHLEBOTOMY | Induction phase 350-500 mL (according to sex/weight) every 1-2 weeks. Check Hb before each phlebotomy (discontinue/delay if <11-12 g/dL) and ferritin every 4 phlebotomies (more frequently when ferritin <200 mg/L). Goal: ferritin ~50 mg/L. Maintenance phase 2-4 phlebotomies per year to keep ferritin within the goal of ~50-100 mg/L. Patients may be usefully enrolled as regular blood donors. Typically well tolerated. Problematic if hemodynamic instability (advanced liver or heart failure), poor venous access, needle phobia, living away from health care facilities. | • LIVER AND HEART FAILURE Erythrocytapheresis preferable over phlebotomy (isovolemic procedure). Deferoxamine in heart failure during the first phases. • HORMONE REPLACEMENT THERAPY (Eg, insulin, androgens) • ARTHROPATHY (May not respond to phlebotomy). Analgesia, physiotherapy, joint replacement • OSTEOPOROSIS Biphosphonates, vitamin D |
| • ERYTHROCYTAPHERESIS | More rapid iron depletion. Cost issues (equipment, personnel). It can preserve blood volume. | ||
| • IRON-CHELATING AGENTSa | Deferoxamine (parenteral), or deferasirox and deferiprone (oral). Use limited to patients with severe life-threatening IO (alone or in combination) or intolerant to phlebotomy. A careful evaluation of the risk-benefit ratio is needed, and they should be prescribed by clinicians with expertise in IO disorders. | ||
| • HEPCIDIN MIMETICS | Rusfertide (weekly SC injections) promising for maintenance phase (needs confirmation). | ||
Lifestyle measures are an integral part of the treatment. Patients are often worried about the need to avoid iron-rich foods, particularly red meat. There is no reason to prescribe drastic diets, as iron is ubiquitous in foods.
SC, subcutaneous.