Transfusional iron overload syndrome

Question 1

Most common settings

Answer 1

• Thalassemia major

- sickle cell

Question 2

Number of transfusions it requires typically

Answer 2

At least 10 (approaching ferritin of 1000)

Question 3

how to quantify tissue iron deposition

Answer 3

Hepatic MRI

Question 4

Other organ to worry about

Answer 4

Cardiac iron overload

Question 5

goal ferritin with treatment

Answer 5

less than 500

Question 6

Treatment for iron overload

Answer 6

Encouraged cessation of alcohol
Patient educated to avoid oral iron ingestion/intake (multivitamins)
IF HgB okay → phlebotomy
IF anemic → Iron chelator: Deferasirox PO or deferoxamine (only IV) + council on GI side effects
IF SCD → exchange transfusion

Question 7

Problem with iron chelators

Answer 7

Relatively toxic and expensive

Question 8

SE’s of iron chelators

Answer 8

Kidney, liver damage, agranulocytosis, ocular and ophthalmic disorders

Question 9

Iron chelator of choice

Answer 9

Deferasirox (oral, deferoxamine is only parenteral)

Question 10

Details to know about deferasirox administration

Answer 10

• Take in the evening before dinner (better tolerated with fewer GI side effects)