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Recurrent paroxysmal cold haemoglobinuria in a 3-year-old child: a case report
C J C Taylor1, J R Neilson, D Chandra
1Medical Department, National Blood Service, Birmingham, UK. craig.taylor@nbs.nhs.uk
Insights
Recurrent paroxysmal cold haemoglobinuria (PCH) in a child is rare. This case highlights a 3-year-old girl experiencing two PCH episodes, emphasizing the need for awareness of this condition.
Area of Science:
- Pediatrics
- Hematology
- Immunology
Background:
- Paroxysmal cold haemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia.
- It is characterized by the rapid destruction of red blood cells upon exposure to cold.
- PCH is typically mediated by Donath-Landsteiner antibodies.
Observation:
- A 3-year-old girl presented with recurrent episodes of PCH.
- Symptoms included malaise, fever, dark urine, and severe anemia following upper respiratory infections.
- She required blood transfusions on both occasions but recovered quickly.
Findings:
- Direct antiglobulin test was positive with anti-C3d.
- Agglutination was observed on the blood film.
- A biphasic Donath-Landsteiner antibody was detected, though its specific target (not anti-P) was unidentified.
Implications:
- This case represents the first reported instance of recurrent acute PCH in a child.
- It underscores the importance of considering PCH in pediatric patients with unexplained hemolytic anemia, especially after infections.
- Further research into the specificities of Donath-Landsteiner antibodies in pediatric PCH is warranted.
Abstract:
We present the case of a 3-year-old girl who presented on two separate occasions, eight months apart, with typical features of paroxysmal cold haemoglobinuria (PCH). On each occasion, she suffered malaise and fever with dark urine following an upper respiratory tract infection. She developed severe anaemia and required transfusion of red cells on both occasions, although each time she made a quick recovery. She was found to be direct antiglobulin test-positive with anti-C3d, and agglutination was observed on the blood film. A biphasic Donath-Landsteiner antibody was demonstrated. The specificity of the antibody was not identified, although it was not anti-P. As far as we are aware, recurrence of acute PCH in a child has not been previously reported in the literature.