Haemoglobin E disease in Bahrain

Abstract EN

-Objective : To report the presence of abn01·mal haemoglobin E (Hb E) in the Bahraini population and study its interaction with other haemoglobinopat.hies prevalent in Bahrain.

Study Design : a) Identification of Hb E during routine haemoglobinopathy screening and subsequent confirmation by various biochemical methods.

b) Analysis of haematological data of all cases of Hb E detected during the year 1997.

Res ults: Seventeen cases (famiJies) ·with Hb E were identified in Bahr·ainis in 1997.

They consisted of 13 cases of Hb E trait, 3 cases of Hb S-E disease and one case of Hb E-Beta thalassaemia.

Haematological data showed normal to mild disease in the t•·ait form and a severe•· picture when in combination with Hb Sand o•· Beta thalassaemia.

Conclusion : This is the first rep01·t of the existence of Hb E in the Bahraini population.

When in combination ""·ith other haemoglobinopathies namely Hb Sand Beta thalassaemia, both of which are highly prevalent in Bah•·ain, it can p1·oduce a clinical disorder, similar to Sickle Cell disease or Beta thalassaem.ia major, requiring dose monitoring, treatment and genetic counselin