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Infectious Disease11 papers

Post-viral paroxysmal cold hemoglobinuria

Last edited: 4/10/2026

Overview

Paroxysmal cold hemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia characterized by the acute release of hemoglobin into the urine following exposure to cold 1. It is typically triggered by cold temperatures and can lead to dark urine, jaundice, and abdominal pain 1.

Diagnosis

  • Diagnosis is suspected in patients with a history of cold exposure followed by symptoms of hemolysis 1.
  • Key diagnostic findings include hemoglobinuria, anemia, and jaundice 1.
  • The Donath-Landsteiner (D-L) test is the definitive diagnostic test, detecting the presence of a specific autoantibody that binds to red blood cells in the cold and activates complement in the presence of warmth 1.

    • Management

  • The primary management strategy is strict avoidance of cold exposure 1.
  • For severe cases, immunosuppressive therapy, such as corticosteroids, may be considered 1.
  • Blood transfusions may be necessary for symptomatic anemia 1.

    • Special Populations

  • PCH can occur in children, often following viral infections 1.

    • Key Recommendations

  • Strict avoidance of cold exposure is the cornerstone of management 1. (Evidence: Moderate)
  • The Donath-Landsteiner test should be performed to confirm the diagnosis 1. (Evidence: Moderate)
  • Immunosuppressive therapy, such as corticosteroids, may be indicated for severe or persistent hemolysis 1. (Evidence: Weak)

    • References

    1 Al-Aly Z, Topol E. Solving the puzzle of Long Covid. Science (New York, N.Y.) 2024. link

    Original source

    1. [1]
      Solving the puzzle of Long Covid. — Al-Aly Z, Topol E Science (New York, N.Y.) (2024)
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