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Types of sickle cell disease seen in the UK

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

  • also for HbS - the person is said to have homozygous SCD (HbSS) (1)

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

    • HbC 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:

  1. Sickle Cell Society. Standards for the clinical care of adults with sickle cell disease in the UK. 2nd edition, 2018
  2. NHS England. SCT screening: handbook for antenatal laboratories. Policy and standards for antenatal laboratories working with the NHS sickle cell and thalassaemia (SCT) screening programme. Published November 2021, last updated October 2024

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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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