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Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update

Paula H B Bolton-Maggs1, Jacob C Langer, Achille Iolascon

  • 1University of Manchester, Manchester, UK. Paula.Bolton-Maggs@manchester.ac.uk

British Journal of Haematology
|November 8, 2011
PubMed

Insights

Updated guidelines for hereditary spherocytosis (HS) management emphasize revised surgical approaches, particularly for children. New insights into red cell membrane biochemistry and diagnostic tests like the eosin-5-maleimide (EMA) binding test are also highlighted.

Area of Science:

  • Hematology
  • Genetics
  • Biochemistry

Background:

  • The 2004 guidelines for hereditary spherocytosis (HS) require updating due to evolving clinical opinions.
  • Recognized long-term risks associated with splenectomy necessitate a review of surgical management protocols.
  • Advances in understanding red cell membrane biochemistry inform diagnostic strategies for HS.

Purpose of the Study:

  • To provide updated clinical guidelines for hereditary spherocytosis management.
  • To reflect current understanding of surgical interventions, including splenectomy and cholecystectomy.
  • To incorporate recent advancements in diagnostic testing for HS.

Main Methods:

  • Review of current clinical opinions on surgical management of HS.
  • Incorporation of new knowledge regarding long-term hazards of splenectomy.
  • Validation of diagnostic tests, including the eosin-5-maleimide (EMA) binding test and biochemical assays.

Main Results:

  • Revised indications for concomitant splenectomy and cholecystectomy in children with mild HS.
  • Updated recommendations for managing asymptomatic gallstones in conjunction with splenectomy.
  • Established diagnostic value of the EMA binding test, acknowledging its limitations.

Conclusions:

  • Current guidelines offer updated recommendations for surgical management of hereditary spherocytosis.
  • Diagnostic approaches are refined, incorporating biochemical and genetic analyses for atypical cases.
  • The EMA binding test is a validated diagnostic tool for hereditary spherocytosis.

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