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Contribution of Genetic Factors in Variation of Clinical Severity Among Siblings with Homozygous β-Thalassemia in Two Indian Families

Abstract We report two Indian families with a variable degree of anemia in two ß thalassemia homozygous siblings from each family. In both the families the siblings with delayed presentation had coinherited an α thalassemia 2 determinant as well as a gene for increased Hb F production. As against this, the severely affected siblings from both the families had shown either absence or presence of only one ameliorating factor. It appears that contribution of two ameliorating factors (α thalassemia 2, Xmn 1 polymorphism) could synergistically compensate for lack of ß globin chains in the homozygotes leading to a milder presentation in the form of thalassemia intermedia.