Thalassemia

Question 1

What is thalassemia major?

Answer 1

An autosomal recessive disorder characterized by the reduced production of 1 of the globin chains in the synthesis of hemoglobin

basically hemoglobin is not adequately made in sufficient amounts because a necessary part of its structure is not produced enough

Question 2

The incidence of thalassemia major is highest in individuals of _______________ descent

Answer 2

Mediterranean

Question 3

What is the goal of Thalassemia major treatment?

Answer 3

Treatment is supportive. The goal of therapy is to maintain normal hemoglobin levels by the administration of blood transfusions.

Question 4

What is the major complication of long term transfusion therapy and how is this addressed ?

Answer 4

Hemosiderosis is a term used for excessive accumulation of iron deposits called hemosiderin in the tissues. The lungs and kidneys are often sites of hemosiderosis.

To prevent organ damage from too much iron, chelation therapy with either Exjade or deferoxamine may be prescribed

Question 5

Deferoxamine is classified as an antidote for acute iron toxicity. TRUE OR FALSE

Answer 5

TRUE

Question 6

Remember to associate defer- with

Answer 6

removal of iron