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Paroxysmal Cold Hemoglobinuria in a 4-year-old Child
Rahul Naithani1, Preethi Jeyaraman2, Bhaskar Saikia3
1Division of Hematology and Bone Marrow Transplantation, Max Superspeciality Hospital, Saket, New Delhi, India. dr_rahul6@hotmail.com.
Indian Pediatrics
|November 15, 2019
Summary
Paroxysmal cold hemoglobinuria (PCH) is a rare condition causing intravascular hemolysis in children after viral infections. This case highlights a self-limiting PCH presentation in a young boy, confirmed by the Donath Landsteiner antibody test.
Area of Science:
- Pediatrics
- Hematology
- Immunology
Background:
- Paroxysmal cold hemoglobinuria (PCH) is a rare autoimmune hemolytic anemia.
- It is characterized by intravascular hemolysis triggered by cold exposure.
- PCH is often associated with viral infections, particularly in pediatric populations.
Observation:
- A 4-year-old boy presented with sudden-onset intravascular hemolysis.
- The patient had a recent history of an acute viral illness.
- Clinical signs of hemolysis were rapidly progressive.
Findings:
- The Donath Landsteiner antibody test was positive, confirming PCH.
- The patient experienced rapid onset of intravascular hemolysis.
- Hemolysis resolved spontaneously within two weeks.
Implications:
- This case underscores the self-limiting nature of PCH in children following viral infections.
- Early diagnosis and supportive care are crucial for managing PCH.
- Understanding the role of the Donath Landsteiner antibody aids in PCH diagnosis.
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