Internal Medicine

0521779407-09 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:12

672 Hemochromatosis

specific therapy

Indications for Treatment

■Remove excess iron if serum ferritin >300μg/L

Treatment Options

■Phlebotomy (treatment of choice): remove 500 mL of blood per week

until ferritin < 50 mcg/L; thereafter, every 3–4 months

■Deferoxamine: with severe cardiac disease or significant anemia,

consider parenteral deferoxamine; inconvenient, expensive, and

removes only 60–80 mg of iron per day.

Side Effects and Contraindications

■Phlebotomy

➣Side effects: anemia

➣Contraindications

Absolute: severe cardiac disease, severe anemia

Relative: hemoglobin <11 g/dL or hematocrit <35%

■Deferoxamine

➣Side effects: flushing, urticaria, hypotension, and shock may

occur. High doses may cause visual disturbances, hearing loss,

respiratory distress syndrome, and death.

➣Contraindications

Absolute: known hypersensitivity

Relative: severe renal failure

follow-up

During Treatment

■Symptoms & hematocrit weekly

■Aim for transferrin saturation <30% and ferritin <50 mcg/L, with

checks every 1–3 months

Routine

■Transferrin saturation and ferritin every 12 months

complications and prognosis

■Cirrhosis: seen in 50–70% of patients with symptoms; screen for HCC

and treat as per liver failure due to other causes; consider liver trans-

plantation for end-stage liver disease

■HCC: risk increased 200-fold in cirrhotic patients; occurs in 25–30%

of cirrhotic individuals; can screen with serum alpha-fetoprotein

and ultrasound every 6 months, but no data proves this improves

outcome