Thalassaemia intermedia in Cyprus: the interaction of α and β thalassaemia

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Abstract
Restriction endonuclease analysis was performed on the .alpha. and .beta. globin gene clusters of 57 Cypriots homozygous for .beta. thalassemia, 30 with the transfusion dependent form of the condition (thalassemia major) and 27 who are less severely affected (thalassemia intermedia). There was a significant difference in the incidence of .alpha. thalassemia between the 2 groups: 14/27 of the patients with thalassemia intermedia also had deletion forms of .alpha. thalassemia, while only 4/30 of the patients with thalassemia major were similarly affected. Thus, in Cypriot patients who are homozygous for .beta. thalassemia the co-inheritance of .alpha. thalassemia is an important factor in determining the clinical course.