Cold agglutinin disease
Description
- Complement-mediated haemolytic anaemia driven by a monoclonal IgM cold agglutinin
- Two clinical problems, and they need different treatment
1. Haemolysis - anaemia, jaundice, fatigue, dark urine in crises
2. Circulatory/agglutination symptoms in the cold - acrocyanosis, Raynaud-like changes, livedo reticularis, cold-induced pain, rarely acral ulceration
| Primary cold agglutinin DISEASE | Secondary cold agglutinin SYNDROME | |
|---|---|---|
| Driver | Clonal bone marrow lymphoproliferative disorder (low-grade, IgM kappa) | **Mycoplasma pneumoniae (anti-I), EBV (anti-i)**, other infection, Waldenstrom/B-cell lymphoma |
| Course | Chronic, indolent, does not remit | Transient, post-infectious - resolves in weeks |
| Treatment | B-cell directed / complement inhibition | Supportive; treat the infection |
- *Not a subtype of warm AIHA managed the same way. Steroids and splenectomy do not work.*
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