Cold Agglutinin Disease

Cold Agglutinin Disease

 

The cold agglutinin syndrome is a form of immune hemolytic anemia.

Epidemiology

  • Incidence – 1/100,000 in U.S.
  • Age – peak onset in 7th decade
  • Gender – equal gender distribution

Risk Factors

  • Presence of lymphoproliferative disorders
  • Infectious process
    • Mycoplasma pneumonia
    • Influenza A and B
    • Adenoviral infection
    • Mononucleosis
    • Malaria
  • Genetic
    • Mutations – Trisomy 3 and 12

Pathophysiology

  • Immunoglobulin-mediated deposition of complement on red cell membranes

Clinical Presentation

  • Hemolytic anemia is often the sole manifestation
  • Acrocyanosis, Raynaud phenomenon
  • Chronic cold agglutinin disease rarely exists

Diagnosis

  • Laboratory testing
    • Serum cold agglutinins
      • Low levels may also be found in healthy adults, peripheral vascular disease, nonlymphoid neoplasm

See Also