Hereditary spherocytosis

Last reviewed: 25 Aug 2023
Last updated: 20 Jun 2023



History and exam

Key diagnostic factors

  • positive family history
  • at risk demographic
  • pallor
  • jaundice
  • splenomegaly
More key diagnostic factors

Other diagnostic factors

  • fatigue
  • hydrops fetalis or stillbirth
Other diagnostic factors

Risk factors

  • family history of splenectomy, anemia, jaundice, or HS
  • at risk demographic
More risk factors

Diagnostic investigations

1st investigations to order

  • CBC
  • reticulocyte count
  • blood smear
  • serum bilirubin
  • serum aminotransferases
  • direct antiglobulin test (DAT)
More 1st investigations to order

Investigations to consider

  • eosin-5-maleimide binding test
  • acidified glycerol lysis test
  • sodium dodecyl sulfate-polyacrylamide gel electrophoresis
More investigations to consider

Emerging tests

  • genetic analysis

Treatment algorithm


neonates (<28 days old)

infants (>28 days old), children, and adults: severe HS

infants (>28 days old), children, and adults: mild-to-moderate HS



Shelley Crary, MD, MSCS

Associate Professor of Pediatrics

University of Arkansas for Medical Sciences

Little Rock



SC is reimbursed for membership on a drug and safety monitoring board (Novartis) for a nonrelated drug.


Dr Shelley Crary would like to gratefully acknowledge Dr Paula Bolton-Maggs, a previous contributor to this topic.


PB-M has received travel and accommodation payments to give a series of lectures on paediatric haematology, one of which was on HS. She also was an expert witness in a legal case concerning a child with HS. PB-M is an author of some references cited in this topic.

Peer reviewers

Robert Schilling, MD

Professor of Medicine Emeritus

School of Medicine and Public Health

University of Wisconsin-Madison




RS is an author of a number of references cited in this topic.

  • Hereditary spherocytosis images
  • Differentials

    • Nonhemolytic anemia
    • Other causes of hemolytic anemia
    • Other causes of spherocytosis
    More Differentials
  • Guidelines

    • Recommendations regarding splenectomy in hereditary hemolytic anemias
    • ICSH guidelines for the laboratory diagnosis of non-immune hereditary red cell membrane disorders
    More Guidelines
  • Patient leaflets

    Jaundice in newborn babies


    More Patient leaflets
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