Haemoglobin H disease and β-thalassaemia: Clinical haematological and electrophoretic studies in a family from South Lebanon

A family is described in which four sibs are affected with haemoglobin H disease. To our knowledge, this is the first instance where this disorder has been encountered in the Lebanon. In fact only a few cases have so far been reported from the Arab world. All four sibs had typical haemoglobin H band...

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Veröffentlicht in:Journal of medical genetics 1974-09, Vol.11 (3), p.275-279
Hauptverfasser: Shahid, Munib J., Khouri, Farid P., Sahli, Itaf F.
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Sprache:eng
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Zusammenfassung:A family is described in which four sibs are affected with haemoglobin H disease. To our knowledge, this is the first instance where this disorder has been encountered in the Lebanon. In fact only a few cases have so far been reported from the Arab world. All four sibs had typical haemoglobin H bands on electrophoretic examination, and characteristic intracorpuscular inclusion bodies were demonstrated in a variable proportion of their erythrocytes, as well as in cells from a younger sib and from the mother. The latter also had elevation of the Hb-A2 fraction, and it is suggested that the above family has a combination of α- and β-thalassaemia.
ISSN:0022-2593
1468-6244
DOI:10.1136/jmg.11.3.275