COLD AGGLUTININ DISEASE
By Gary M. White, MD
Cold agglutinin disease is a rare form of autoimmune hemolytic anemia in which exposure to cold triggers thrombi formation.
- V4-34 gene segment–encoded autoantibodies, usually directed at I and/or i carbohydrate antigens on the surface of erythrocytes may be found.
- Diagnosis is made with a positive cold agglutinin titer.
The ears and other acral sites become red and inflamed upon exposure to the cold.
Cold avoidance is key. Systemic steroids have been used as have cytotoxic agents, plasmapheresis and rituximab.
Red, inflamed ears after a day exposed to the cold on a boat. JAMA Derm 2015;151;785.
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