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Thalassemia

 

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SUSAN LECLAIR

Edna - Thalassemia does NOT cause iron deficiency. If anything it is association with iron OVERLOAD.
A little background - hemoglobin is made up of 4 protein chains and embedded in each chain is a heme molecule which is a protoporphyrin and iron complex. (Think of it as a diamond iron with the protein chains as the ring, the protoporphyrin is the setting and the iron is the jewel.) Thalassemia is an inherited disorder in which either 1) you are missing part of the gene that "makes" hemoglobin chains or 2) you are making the chains at a much slower than normal rate. The end presentation in the red cell is a lack of hemoglobin which many people think is a lack of iron - which it is not. They have plenty of iron but it cannot be used in hemoglobin since they only make less than 40% (in the most severe case) to about 80% (in the milder forms) of the "normal" amount of hemoglobin.
While one form of Thalassemia was found in people of Mediterranean descent, there are actually 7 different types of Thalassemia - each with its own severe/moderate/mild presentations. Beta thalassemia (the original Mediterranean anaemia) can be found in people in the Philippines and Micronesia as well as people from the Mediterranean while alpha thalassemia has been found in people from Northern Europe. It sounds as if you have one of the milder forms which has escaped detection in earlier generations because of its mildness
 

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