© 2004 BMJ Publishing Group & Royal College of Paediatrics and Child Health
LEADING ARTICLE
New guidelines
Hereditary spherocytosis; new guidelines
Correspondence to:
Correspondence to:
Dr P Bolton-Maggs
Consultant Haematologist, Department of Clinical Haematology, Manchester Royal Infirmary, Oxford Road, Manchester M13 9WL, UK; paula.bolton-maggs@man.ac.uk
Accepted 24 February 2004
Hereditary spherocytosis
Keywords: hereditary spherocytosis; splenectomy; prophylaxis
| The first 150 words of the full text of this article appear below. |
Hereditary spherocytosis (HS) is the commonest cause of haemolysis in northern Europe. Most children have mild disease with little interference with lifestyle. Presentation with parvovirus B19 infection causing transient severe anaemia is not uncommon. The laboratory diagnosis of HS is usually straightforward and additional tests are rarely required. A new test, EMA binding, will probably replace the time consuming and non-specific osmotic fragility test. Splenectomy leads to improved red cell survival and is indicated for severe and probably moderate disease; gallstones may occur in the first decade, and if symptomatic are an indication for both cholecystectomy and splenectomy. Splenectomy is associated with a life long increased risk of sepsis, which has not been completely eradicated by preoperative vaccinations and post-splenectomy antibiotic prophylaxis.
Haemolysis is an uncommon cause of anaemia in children. The commonest cause of anaemia is iron deficiency, globally a major problem; worldwide, haemolysis is most commonly
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