Cold agglutinin disease

Cold agglutin disease

The clumps of red cells seen here are typical of this disorder.

Key features include:

  • Autoimmune condition causing haemolysis
  • The auto-antibody coating the red cells are IgM (can be poly or monoclonal)
  • Monoclonal antibodies are directed at the I/i red cell antigen
  • The direct antiglutinin test is usually positive for C3d
  • At body core temperature, the antibody is unbound
  • In the cooler peripheries the antibody binds to red cells and induces the complement cascade inducing haemolysis
  • Patients respond poorly to corticosteroids
  • Management is usually supportive (transfusion through a blood 
  • warmer) but Rituximab, Fludarabine and even plasma exchange can be used
  • Remember-keep the patient warm!


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