thalassemia

The anemias known as the thalassemias are inherited disorders of hemoglobin production. In these conditions production of the protein portions (globin) of the hemoglobin molecule is decreased, and the globin protein itself is abnormal. The red cells thus produced are smaller ("microcytic") and more pale than normal ("hypochromic"), and are more fragile and prone to early destruction.

There are two different chains of protein in the hemoglobin molecule, alpha and beta, and either can be affected. There are at last count over 100 different mutations that can cause thalassemia, so the diseases are variable and somewhat confusing; however they can be classified into two main groups: alpha and beta thalassemias, according to which of the two globin chains are defective.

Thalassemia genes are remarkably widespread in the peoples native to the areas bordering the Mediterranean Sea, much of Africa, the Middle East, the Indian subcontinent, and Southeast Asia - wherever malaria is prevalent. The carrier state of thalassemia genes confers resistance to lethal malarial infections in much the same way as the carrier state for sickle cell anemia, and this fact accounts for the prevalence of the genes.

Carriers of thalassemia genes usually are identified by a mild anemia masquerading initially as iron deficiency. These patients do not respond to iron therapy, and sometimes the parents are suspected of not giving the iron properly. A hemoglobin anaylsis (electrophoresis) test may be ordered to clarify the diagnosis, and to prevent future confusion and needless iron therapy.

Thallasemia major - the disease produced when an individual carries two genes for the defective hemoglobin - is a much more serious condition. Patients with thalassemia major (Cooley's anemia) have a much more serious illness, which requires lifelong transfusion therapy and nightly iron chelation treatments to fight iron overload from the numerous blood transfusions.

The Cooley's Anemia Foundation is the nation's leading organization fighting thalassemia. Much more information about thalassemia can be obtained at their web site.

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