What is the treatment for high hematocrit (elevated red blood cell count) with high ferritin (iron overload)?

Treatment for High Hematocrit with High Ferritin

Phlebotomy is the first-line treatment for patients with high hematocrit and high ferritin levels, with a target ferritin level of 50-100 μg/L to effectively reduce iron overload and improve clinical outcomes. 1

Initial Treatment Phase

• Weekly or biweekly phlebotomy (removal of one unit of blood, approximately 500 mL) should be performed during the initial iron depletion phase to effectively reduce iron overload 1, 2
• Hemoglobin should be checked before each phlebotomy session:
  • If <12 g/dL: reduce frequency of phlebotomy
  • If <11 g/dL: temporarily pause treatment 1
• Monitor serum ferritin after every 4 phlebotomies until reaching 200 μg/L, then check ferritin every 1-2 treatment sessions 1
• The initial target ferritin level is 50 μg/L 1
• The number of phlebotomies required correlates strongly with the initial ferritin level (higher initial ferritin requires more phlebotomy sessions) 3

Maintenance Phase

• Once the target ferritin level is reached, transition to maintenance phlebotomy (typically 2-6 sessions per year) to maintain ferritin between 50-100 μg/L 1, 2
• Continue to check hemoglobin before each phlebotomy session 1
• Monitor ferritin and transferrin saturation every 6 months during maintenance 1
• Lifelong follow-up is necessary to prevent reaccumulation of iron 1, 2

Alternative Treatment Options

• Erythrocytapheresis can be considered as an alternative to phlebotomy in selected cases:

  • Allows for fewer hemodynamic changes compared to phlebotomy
  • Results in fewer total procedures and shorter treatment duration
  • Particularly beneficial for patients who poorly tolerate standard phlebotomy 1, 4
  • However, availability may be limited and costs can be higher 1

• Iron chelation therapy (e.g., deferasirox) should only be considered as a second-line option when phlebotomy or erythrocytapheresis is not feasible:

  • Only after careful risk-benefit assessment
  • Not approved by European Medicines Agency for hemochromatosis
  • Associated with potential side effects including gastrointestinal issues and kidney impairment 1, 5

Clinical Benefits of Treatment

• Effective iron removal through phlebotomy can improve:
  • Fatigue and malaise
  • Skin pigmentation
  • Insulin requirements in diabetics
  • Abdominal pain
  • Liver enzyme levels 1
• Treatment may also prevent progression to more serious complications such as:
  • Hepatic cirrhosis
  • Primary liver cancer
  • Diabetes mellitus
  • Cardiomyopathy 1, 6

Special Considerations

• Avoid vitamin C supplements during treatment as they can accelerate iron mobilization to potentially dangerous levels 1, 2
• Dietary modifications alone are insufficient to treat iron overload but avoiding iron supplements, vitamin C supplements, and excessive red meat consumption is recommended 1
• Proton pump inhibitors (when prescribed for other indications) may reduce phlebotomy requirements 1
• In patients with both high hematocrit and high ferritin due to cyanotic heart disease or chronic hypoxia, a combination of phlebotomy with iron therapy may be needed to improve microcytic polycythemia while maintaining stable hematocrit 7, 8

Monitoring and Follow-up

• Regular monitoring of hemoglobin, ferritin, and transferrin saturation is essential throughout treatment 1
• Liver function should be monitored as improvement in liver enzymes is often seen with iron depletion 3
• The development of iron deficiency, anemia, or an unexplained reduction in phlebotomy requirements should prompt investigation for alternative causes 1