Thalassemia

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Thalassemia major

Thalassemia

Alternative Names:

Thalassemia; Mediterranean anemia; Cooley's anemia
Treatment:

With severe thalassemia, regular blood transfusions and folate supplementation are given. People who receive the blood transfusions should avoid iron supplements and oxidative drugs such as sulfonamides, because iron levels can become toxic.

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Patients who receive significant numbers of blood transfusions require therapy to remove iron from the body, called chelation therapy. Bone marrow transplant is being investigated as a treatment and is most successful in children.

Expectations (prognosis):

In severe thalassemia, death from heart failure can occur between the ages of 20 and 30. Hypertransfusion programs with chelation therapy improve outcome, and successful bone marrow transplantation is curative. Less severe forms of thalassemia usually do not shorten life span.

Complications:

Untreated, thalassemia major leads to heart failure as well as liver dysfunction, and susceptibility to infection.

Iron overload as a side effect of treatment can cause damage to the heart, liver, and endocrine systems. This complication is managed by daily injections of an iron chelating agent, which binds to iron and causes it to be excreted in the urine.

Calling your health care provider:

Call for an appointment with your health care provider if symptoms develop that are suggestive of thalassemia.

Call your health care provider if symptoms develop after treatment.