Sickle cell anemia

Last reviewed: 24 Aug 2023
Last updated: 17 Jan 2023



History and exam

Key diagnostic factors

  • parent(s) diagnosed with sickle cell anemia, other sickle cell disease, or sickle cell trait
  • persistent pain in skeleton, chest, and/or abdomen
  • dactylitis
More key diagnostic factors

Other diagnostic factors

  • high temperature
  • pneumonia-like syndrome
  • bone pain
  • visual floaters
  • tachypnea
  • failure to thrive
  • pallor
  • jaundice
  • tachycardia
  • lethargy
  • protuberant abdomen, often with umbilical hernia
  • cardiac systolic flow murmur
  • maxillary hypertrophy with overbite
  • shock
Other diagnostic factors

Risk factors

  • genetic
More risk factors

Diagnostic investigations

1st investigations to order

  • DNA-based assays
  • hemoglobin isoelectric focusing (Hb IEF)
  • cellulose acetate electrophoresis
  • high-performance liquid chromatography (HPLC) fractionation
  • hemoglobin solubility testing
  • peripheral blood smear
  • CBC and reticulocyte count
  • iron studies
More 1st investigations to order

Investigations to consider

  • pulse oximetry
  • plain x-rays of long bones
  • bacterial cultures
  • chest x-ray
More investigations to consider

Treatment algorithm


vaso-occlusive crisis

acute chest syndrome


chronic disease



Sophie Lanzkron, MD, MHS


Sickle Cell Center for Adults

Associate Professor of Medicine and Oncology

Johns Hopkins Medicine




SL has received funds for consultancy work for Bluebird Bio, Novartis, Novo Nordisk, and Pfizer. SL has stock interest in Pfizer and Teva. SL’s institution has received research funding from Novartis, Imara, Shire, GBT, CSL Behring, University of Pittsburgh, National Heart Lung and Blood Institute, Patient-Centered Outcomes Research Institute, and Health Resources and Services Administration.


Dr Sophie Lanzkron would like to gratefully acknowledge Dr Channing Paller, a previous contributor to this topic.


CP declares that she has no competing interests.

Peer reviewers

James Bradner, MD

Instructor in Medicine

Division of Hematologic Neoplasia

Dana-Farber Cancer Institute




JB declares that he has no competing interests.

Adrian Stephens, MB BS, MD, FRCPath

Consultant Haematologist

University College London Hospitals




AS declares that he has no competing interests.

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