Last reviewed: September 2018
Last updated: August  2018

Summary

Definition

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 (Hb 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
  • chest x-ray
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Director

Sickle Cell Center for Adults

Associate Professor of Medicine and Oncology

Johns Hopkins Medicine

Baltimore

MD

Disclosures

SL has been the site principal investigator on several industry-funded studies with the following companies: Pfizer, Selexys, Astra Zeneca, and Prolong. She also receives research funding from PCORI and HRSA.

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 VIEW ALL

Instructor in Medicine

Division of Hematologic Neoplasia

Dana-Farber Cancer Institute

Boston

MA

Disclosures

JB declares that he has no competing interests.

Consultant Haematologist

University College London Hospitals

London

UK

Disclosures

AS declares that he has no competing interests.

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