Sickle cell disease in adult bedouins of Al-Ain district, UAE

ABSTRACT

Twenty-four adult UAE national and Omani patients with sickle cell disease were diagnosed during the period 1987 - 1991 in Al-Ain Hospital, United Arab Emirates. Their clinical, haematological and biochemical parameters were analysed. The overall picture was that of a mild-to-moderate disease similar to that described in Eastern Saudi Arabia and different from the severe form seen in Africa. Most patients had few admissions, vaso-occlusive crises, blood transfusions or other complications. Eleven patients had homozygous sickle cell disease [HbSS] and 13 were heterozygous for sickle cell and beta thalassaemia. In general, patients with homozygous sickle cell disease manifested a similar clinical disease pattern and a similar degree of severity to that of patients who had associated thalassaemia. HbF was elevated in all patients, with no difference between the HbSS and the Sickle Cell/beta thalassaemia groups. The levels of HbF in both groups did not relate to the severity of the disease