Current Paediatrics
Volume 12, Issue 4 , Pages 290-297, August 2002

The management of haemoglobinopathies

  • Manoj Raghavan (Specialist Registrar in Haematology)

      Affiliations

    • Department of Haematology, Great Ormond Street, Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK
    • ,
  • Sally C. Davies (Consultant Haematologist)

      Affiliations

    • Imperial College Faculty of Medicine at Central Middlesex Hospital, Acton Lane, Park Royal, London, NW10 7NS, UK
    • Correspondence to: SCD. Imperial College Faculty of Medicine at Central Middlesex Hospital, Acton Lane, Park Royal, London NW10 7NS, UK. Tel.: +44 (0)20 7725 5408; Fax: +44 (0)20 7725 5467; E-mail: sally.davies@doh.gsi.gov.uk

Abstract 

Sickle cell disease (SCD) and thalassaemia major are the most important syndromes of the haemoglobinopathies. Complications of SCD can be life threatening and include stroke, hyposplenism and vaso-occlusive crises. Treatment of SCD is generally supportive or by transfusion, hydroxyurea or bone marrow transplant (BMT). β Thalassaemia major is treated with transfusion and chelation for the resulting iron overload which is the cause of most morbidity, with BMT the only curative procedure.

Keywords: haemoglobinopathies, Sickle cell, thalassaemia, Clinical Management, transfusion, hydroxyurea

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PII: S0957-5839(02)90301-5

doi:10.1054/cupe.2002.0301

Current Paediatrics
Volume 12, Issue 4 , Pages 290-297, August 2002

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