First-line treatment for hereditary hemochromatosis is what?

First-line treatment for hereditary hemochromatosis is what?

• A. Chelation therapy with deferoxamine
• B. High-iron diet
• C. Immunosuppressive therapy
• D. Weekly phlebotomy

Answer: (D)

Hereditary hemochromatosis causes iron overload because of increased intestinal iron absorption. The treatment goal is to lower the body's iron stores to prevent organ damage, especially in the liver, pancreas, and heart. Removing blood through therapeutic phlebotomy directly reduces iron load, since each unit of blood contains a large amount of iron. With regular phlebotomy, ferritin and transferrin saturation fall, iron deposition in organs slows or reverses, and the risk of cirrhosis, diabetes, and cardiomyopathy decreases. In practice, treatment starts with weekly phlebotomy until iron stores are sufficiently depleted (ferritin often guided toward a low-normal range, around 50–100 ng/mL, with normalized transferrin saturation), followed by maintenance phlebotomy every few months to keep iron levels in check. Chelation therapy with deferoxamine is reserved for patients who cannot undergo phlebotomy or in certain forms of iron overload, but it is not the preferred first-line approach in classic hereditary hemochromatosis. A high-iron diet would worsen the problem, and immunosuppressive therapy has no role here.

Hereditary hemochromatosis causes iron overload because of increased intestinal iron absorption. The treatment goal is to lower the body's iron stores to prevent organ damage, especially in the liver, pancreas, and heart. Removing blood through therapeutic phlebotomy directly reduces iron load, since each unit of blood contains a large amount of iron. With regular phlebotomy, ferritin and transferrin saturation fall, iron deposition in organs slows or reverses, and the risk of cirrhosis, diabetes, and cardiomyopathy decreases. In practice, treatment starts with weekly phlebotomy until iron stores are sufficiently depleted (ferritin often guided toward a low-normal range, around 50–100 ng/mL, with normalized transferrin saturation), followed by maintenance phlebotomy every few months to keep iron levels in check.

Chelation therapy with deferoxamine is reserved for patients who cannot undergo phlebotomy or in certain forms of iron overload, but it is not the preferred first-line approach in classic hereditary hemochromatosis. A high-iron diet would worsen the problem, and immunosuppressive therapy has no role here.