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Unusual splenic sinusoidal iron overload in sickle cell/haemoglobin D-Punjab disease
  1. P M C Jiskoot1,
  2. C Halsey2,
  3. R Rivers3,
  4. B J Bain4,
  5. B S Wilkins5
  1. 1Department of Histopathology, St Mary’s Hospital, Praed Street, London W2 1NY, UK
  2. 2Department of Haematology, St Mary’s Hospital
  3. 3Department of Paediatrics, St Mary’s Hospital
  4. 4St Mary’s Hospital Campus of Imperial College Faculty of Medicine, London W2 1NY, UK
  5. 5Department of Histopathology, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK
  1. Correspondence to:
 Dr B S Wilkins
 Department of Histopathology, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK;


Sickle cell/haemoglobin D-Punjab disease is a disorder with similar clinical features to sickle cell anaemia. This report describes the case of an 11 year old boy with this disease who was treated with regular transfusions from infancy. He underwent splenectomy at the age of 10 years for hypersplenism. Histology of the spleen revealed a striking pattern of heavy sinusoidal endothelial iron loading, with only moderate uptake by macrophages. Possible explanations for this unusual distribution of iron include phagocytosis of sickled erythrocytes by sinusoidal endothelial cells or direct endothelial iron uptake via transferrin receptors. Transfusion programmes ameliorate the symptoms of sickle cell disease but the dangers of iron overload should always be remembered.

  • sickle cell/haemoglobin D-Punjab disease
  • sickle cell disease
  • spleen
  • sinusoid
  • endothelium
  • iron overload
  • SD, sickle cell/haemoglobin D-Punjab

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