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Thalassaemia intermedia in Cyprus: the interaction of α and β thalassaemia
Author(s) -
Wainscoat J. S.,
Kanavakis E.,
Wood W. G.,
Letsky E. A.,
Huehns E. R.,
Marsh G. W.,
Higgs D. R.,
Clegg J. B.,
Weatherall D. J.
Publication year - 1983
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1983.00353.x-i1
Subject(s) - intermedia , medicine , hemoglobinopathy , thalassemia , hemolytic anemia , gastroenterology , pediatrics , genetics , biology , history , performance art , art history
S ummary . Restriction endonuclease analysis has been performed on the α and β globin gene clusters of 57 Cypriots homozygous for β thalassaemia, 30 with the transfusion dependent form of the condition (thalassaemia major) and 27 who are less severely affected (thalassaemia intermedia). There was a significant difference in the incidence of α thalassaemia between the two groups: 14/27 of the patients with thalassaemia intermedia also had deletion forms of a thalassaemia, while only 4/30 of the patients with thalassaemia major were similarly affected. Thus in Cypriot patients who are homozygous for β thalassaemia the co‐inheritance of a thalassaemia is an important factor in determining the clinical course.