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

Abstract

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.