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Types of sickle cell disease seen in the UK

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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In patients with sickle cell disease (SCD) there is inheritance of the gene for sickle haemoglobin (Hb S) from one parent and a gene for an abnormal haemoglobin variant from the other parent. If the second abnormal gene is:

  • also for Hb S - the person is said to have homozygos SCD (HbSS) (1)

  • a different abnormal haemoglobin gene - is called compound heterozygous SCD e.g. -

    • Hb C in combination with HbS causes Hb SC sickle cell disease

    • Hb DPunjab in combination with HbS causes Hb SD sickle cell disease

    • Hb OArab - in combination with HbS causes Hb SO sickle cell disease

    • beta thalassaemia in combination with HbS causes Hb S/beta-thalassaemia sickle cell disease

    • Hereditary Persistence of Fetal Haemoglobin in combination with HbS causes Hb S/HPFH sickle cell disease (2)

Reference:

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