What is the management of hereditary hemochromatosis (HH) with concomitant Chronic Kidney Disease (CKD)?

From the Guidelines

Management of hereditary hemochromatosis (HH) in patients with concomitant Chronic Kidney Disease (CKD) requires careful consideration of iron removal while preserving renal function, and the primary approach is therapeutic phlebotomy, with adjustments for CKD patients, especially those with anemia. The treatment of HH in CKD patients involves:

• Therapeutic phlebotomy, removing 400-500 mL of blood every 1-2 weeks until serum ferritin levels reach 50-100 ng/mL, followed by maintenance phlebotomy 2-4 times yearly, with reduced frequency in CKD patients to avoid exacerbating anemia 1.
• Iron chelation therapy as an alternative for patients with severe CKD or those on dialysis who cannot tolerate phlebotomy, with Deferasirox (starting at 10-20 mg/kg/day) being generally preferred but requiring dose reduction in CKD (typically by 50% in moderate CKD) 1.
• Regular monitoring, including monthly complete blood counts, quarterly ferritin and transferrin saturation measurements, and renal function tests 1.
• Dietary modifications to limit iron-rich foods, vitamin C supplements, and alcohol 1. Key considerations in managing HH with CKD include:
• Avoiding iron deficiency while removing excess iron, as CKD patients often have functional iron deficiency despite iron overload 1.
• Monitoring for signs of iron deficiency, such as a decrease in serum ferritin or an increase in hypochromic red blood cells 1.
• Adjusting the phlebotomy frequency and iron chelation therapy based on the patient's renal function and anemia status 1.

From the FDA Drug Label

Deferasirox tablet is contraindicated in adult and pediatric patients with eGFR less than 40 mL/minute/1.73 m2. For patients with renal impairment (eGFR 40 to 60 mL/min/1.73 m2), reduce the starting dose by 50% Exercise caution in pediatric patients with eGFR between 40 and 60 mL/minute/1.73 m2. If treatment is needed, use the minimum effective dose and monitor renal function frequently.

The management of hereditary hemochromatosis (HH) with concomitant Chronic Kidney Disease (CKD) involves reducing the starting dose of deferasirox by 50% in patients with eGFR 40 to 60 mL/min/1.73 m2, and avoiding use in patients with eGFR less than 40 mL/min/1.73 m2.

• Dose reduction: Reduce the starting dose by 50% in patients with eGFR 40 to 60 mL/min/1.73 m2.
• Monitoring: Monitor renal function frequently in patients with CKD.
• Contraindication: Avoid use in patients with eGFR less than 40 mL/min/1.73 m2. 2

From the Research

Management of Hereditary Hemochromatosis (HH) with Concomitant Chronic Kidney Disease (CKD)

• The management of HH with concomitant CKD is complex and requires careful consideration of the patient's iron status, kidney function, and overall health 3, 4.
• Phlebotomy is a common treatment for HH, but it may not be suitable for patients with CKD, as it can lead to anemia and worsen kidney function 5, 3.
• Iron chelation therapy is an alternative treatment option for patients with HH and CKD, and can help to reduce iron overload and prevent organ damage 5, 6.
• Deferasirox, an oral iron chelator, has been shown to be effective in reducing iron overload in patients with HH and CKD, but it may increase the risk of renal or hepatic failure 5.
• The use of erythropoiesis-stimulating agents (ESAs) and iron supplements may be necessary to manage anemia in patients with CKD, but these treatments can also contribute to iron overload and worsen HH 7, 4.
• Close monitoring of iron status, kidney function, and overall health is crucial in the management of HH with concomitant CKD, and treatment should be individualized based on the patient's specific needs and circumstances 3, 4.

Treatment Options

• Phlebotomy: may not be suitable for patients with CKD, as it can lead to anemia and worsen kidney function 5, 3.
• Iron chelation therapy: can help to reduce iron overload and prevent organ damage, but may increase the risk of renal or hepatic failure 5, 6.
• Deferasirox: an oral iron chelator that has been shown to be effective in reducing iron overload in patients with HH and CKD, but may increase the risk of renal or hepatic failure 5.
• ESAs and iron supplements: may be necessary to manage anemia in patients with CKD, but can also contribute to iron overload and worsen HH 7, 4.

Considerations

• The patient's iron status, kidney function, and overall health should be carefully monitored and considered when developing a treatment plan for HH with concomitant CKD 3, 4.
• Treatment should be individualized based on the patient's specific needs and circumstances, and may involve a combination of phlebotomy, iron chelation therapy, ESAs, and iron supplements 3, 4.
• The potential risks and benefits of each treatment option should be carefully weighed, and the patient should be closely monitored for any adverse effects or changes in their condition 5, 6.