Management of cold agglutination syndrome

Cold haemagglutination syndrome is difficult to treat. Fortunately it seldom needs treating. In most cases cold agglutinins are an incidental finding representing either normality or a benign chronic monoclonal gammopathy that does not cause ill health. Two sorts of symptoms are likely in the more severe cases. Acrocyanosis is usually treated by keeping the patient warm and if necessary removing him or her to Florida or the Canary Islands. In the rare cases of haemolytic anaemia, an underlying lymphoid tumour should be sought and treated. If none exists, then it is unlikely that the treatments that are useful in warm antibody haemolytic anaemia will be helpful. Plasma exchange ought to work but in practice there are frequently problems of red cell agglutination within the cell separator or the plastic tubes. For this reason plasma exchange within a heated room is advocated. When cardiac surgery is contemplated pre-operative plasma exchange is sometimes helpful, or the heart may be stopped by potassium solutions and the operation is carried out in the warm.