Hematological Characteristics of Sardinian α-Thalassemia Carriers Detected in a Population Study

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Abstract
88 adults with thalasseia-like red cell indices, normal serum iron and normal hemoglobin (Hb) A2 and F levels, diagnosed in a mass screening had Hb H inclusion bodies studies (65 subjects) or Hb H inclusion bodies studies and globin chain synthesis analysis (23 subjects). The α/β ratio of 0.70 + 0.10 was the same as in obligate α-thalassemia-1 (α-thal) carriers. Hb H inclusion bodies studies were found to be a reliable test for α-thal trait identification, resulting positive in approximately 70% of suspected carriers. The α-thal carrier defined by Hb H preparation or by globin chain synthesis had significant reduction in the mean Hb level, hematocrit, mean corpuscular hemoglobin and a significant increase in mean red cell count, but there was some overlap with controls.

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