How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)

Arch Dis Child Educ Pract Ed. 2022 Jun;107(3):199-206. doi: 10.1136/archdischild-2020-319568. Epub 2021 Aug 25.

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.

Keywords: biochemistry; jaundice; pathology; physiology.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Child, Preschool
  • Cold Temperature
  • Hemoglobinuria, Paroxysmal* / diagnosis
  • Humans
  • Male