RT Journal Article
SR Electronic
T1 Unusual splenic sinusoidal iron overload in sickle cell/haemoglobin D-Punjab disease
JF Journal of Clinical Pathology
JO J Clin Pathol
FD BMJ Publishing Group Ltd and Association of Clinical Pathologists
SP 539
OP 540
DO 10.1136/jcp.2002.004481
VO 57
IS 5
A1 Jiskoot, P M C
A1 Halsey, C
A1 Rivers, R
A1 Bain, B J
A1 Wilkins, B S
YR 2004
UL http://jcp.bmj.com/content/57/5/539.abstract
AB 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.