DOI: 10.36347/sasjm.2024.v10i06.005

Pages: 503-505

Sickle cell disease is an autosomal recessive genetic disorder and one of the most common haemoglobinopathies in Morocco. It is characterised by the polymerisation of haemoglobin S, resulting in a change in the spatial conformation of sickle cell haemoglobin, and consequently in its function. Hydroxyurea is the only product with proven efficacy in preventing the complications of sickle cell anaemia and has been approved by the "Food and Drug Administration". The effects of hydroxyurea are essentially linked to the increase in HbF by inhibiting the polymerisation of haemoglobin S, which is the pathophysiological basis of sickle cell disease. It reduces the frequency of painful attacks in most patients, acute chest syndromes and transfusion requirements in sickle cell patients with a severe form of the disease and prolongs their life expectancy. This was a 40-year-old male patient from a marriage in which the diagnosis of homozygous sickle cell anaemia SS was made following an electrophoresis that showed no trace of foetal haemoglobin, and the patient was put on antibiotics and folic acid without transfusion. The patient was subsequently started on hydroxyurea, 1 x 500 mg tablet twice daily. After 14 years on hydroxyurea, he had no vasoocclusive attacks or acute chest syndrome, and his haemoglobin electrophoresis was as follows: hbF: 42.5% hbS: 54.3% and hbA2: 3.2%. Haemoglobin electrophoresis is a major biochemical examination for monitoring the evolution of haemoglobin S and haemoglobin F fractions in our patient treated with hydroxyurea, a molecule that occupies a privileged place in the management of severe forms of sickle cell disease and gives the best results in the prevention of vaso-occlusive crises and acute thoracic syndromes.