THALASSEMIA IN "NON-MEDITERRANEAN" FAMILIES

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Abstract
A diagnosis of thalassemia is readily entertained in an anemic individual of Italian or Greek extraction but rarely in the absence of such a racial background. It is our impression, however, that minor and intermediate forms of thalassemia are much more common in "non-Mediterranean" families than heretofore recognized. This impression is based on the fact that during the past year, working in a general hospital, we have diagnosed thalassemia in 6 patients of various "non-Mediterranean" backgrounds including Danish, English, Scottish, Filipino and Negro. In each instance there were classic findings of hypochromic, microcytic anemia, targeting, decreased red cell fragility, high serum iron and erythroid hyperplasia of the bone marrow. All patients were studied with regard to red cell survival and ferrokinetics and showed abnormalities consistent with the severity of the clinical picture. Hemoglobin A2, as determined by starch block electrophoresis, was invariably elevated. Family studies were possible in most cases and also supported the diagnosis of heterozygous thalassemia. Our findings clearly indicate that thalassemia should be considered in every patient with hypochromic anemia regardless of racial background since such cases, if unrecognized, may have serious implications from the standpoint of iron overloading.
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