RT Journal Article
SR Electronic
T1 How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)
JF Archives of disease in childhood - Education & practice edition
JO Arch Dis Child Educ Pract Ed
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP 199
OP 206
DO 10.1136/archdischild-2020-319568
VO 107
IS 3
A1 Jennifer Delun Williams
A1 Ram K Jayaprakash
A1 Heena Kithany
A1 Mark Peter Tighe
YR 2022
UL http://ep.bmj.com/content/107/3/199.abstract
AB 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.