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How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)
  1. Jennifer Delun Williams1,
  2. Ram K Jayaprakash2,
  3. Heena Kithany1,
  4. Mark Peter Tighe1
  1. 1 Paediatrics, University Hospitals Dorset NHS Foundation Trust, Poole, Dorset, UK
  2. 2 Haematology, University Hospitals Dorset NHS Foundation Trust, Poole, Dorset, UK
  1. Correspondence to Dr Mark Peter Tighe, Paediatrics, University Hospitals Dorset NHS Foundation Trust, Poole BH15 2JB, Bournemouth Christchurch, UK; mpt195{at}hotmail.com

Abstract

Paroxysmal cold haemoglobinuria (PCH) accounts for around a third of cases of autoimmune haemolytic anaemia in children. PCH is caused by an autoantibody that fixes complement to red cells at low temperatures and dissociates at warmer temperatures (a biphasic haemolysin), triggering complement-mediated intravascular haemolysis. Named the Donath-Landsteiner (D-L) antibody after its discoverers, it is usually formed in response to infection and demonstrates specificity for the ubiquitous red cell P-antigen. A D-L test can be used to detect the presence of the D-L autoantibody in the patients’ serum. Here we discuss the use of the D-L test in identifying PCH in a 2-year-old boy who presented with haemolytic anaemia. A summary of the key information can be found in the infographic.

  • biochemistry
  • jaundice
  • physiology
  • pathology
http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/archdischild-2020-319568

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    Footnotes

    • Contributors JDW, HK and MPT conceived the article. JDW wrote the article, with assistance from HK and MPT, and edited the article for publication. RKJ gave haematological advice. All authors are accountable for this work.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.