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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