Last reviewed: June 2018
Last updated: November  2017



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

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

Diagnostic investigations

1st investigations to order

  • DNA-based assays
  • hemoglobin isoelectric focusing (IEF)
  • cellulose acetate electrophoresis
  • high-performance liquid chromatography (HPLC) fractionation
  • hemoglobin solubility testing
  • peripheral blood smear
  • CBC and reticulocyte count
  • iron studies
Full details

Investigations to consider

  • pulse oximetry
  • plain x-rays of long bones
  • bacterial cultures
  • CXR
Full details

Treatment algorithm


Authors VIEW ALL


Sickle Cell Center for Adults

Associate Professor of Medicine and Oncology

Johns Hopkins Medicine




SL is a paid member of the advisory boards of Global Blood Therapeutics and ApoPharma; her institution has received research funding on multicenter trials from Pfizer, Selexys, Prolong, NKT Therapeutics, and Astra-Zeneca; and she has also received research funding from PCORI, HRSA, and NHLBI.

Dr Sophie Lanzkron would like to gratefully acknowledge Dr Channing Paller, a previous contributor to this monograph. CP declares that she has no competing interests.

Peer reviewers VIEW ALL

Instructor in Medicine

Division of Hematologic Neoplasia

Dana-Farber Cancer Institute




JB declares that he has no competing interests.

Consultant Haematologist

University College London Hospitals




AS declares that he has no competing interests.

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