Last reviewed: November 2017
Last updated: November  2017

Summary

Definition

History and exam

Key diagnostic factors

  • parent(s) diagnosed with sickle cell anaemia, 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
  • tachypnoea
  • failure to thrive
  • pallor
  • jaundice
  • tachycardia
  • lethargy
  • maxillary hypertrophy with overbite
  • protuberant abdomen, often with umbilical hernia
  • cardiac systolic flow murmur
  • shock

Diagnostic investigations

1st investigations to order

  • DNA-based assays
  • haemoglobin isoelectric focusing (IEF)
  • cellulose acetate electrophoresis
  • high-performance liquid chromatography (HPLC)
  • haemoglobin solubility testing
  • peripheral blood smear
  • FBC 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

Contributors

Authors VIEW ALL

Director

Sickle Cell Center for Adults

Associate Professor of Medicine and Oncology

Johns Hopkins Medicine

Baltimore

MD

Disclosures

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

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.

Use of this content is subject to our disclaimer