Cold agglutinin related acrocyanosis and paroxysmal haemolysis.

The clinical features of severe cold agglutinin related paroxysmal agglutination and haemolysis could be confused with a number of conditions. A 61-year old lady presented with features of acute peripheral ischaemia who had recently been started on treatment for Raynaud's disease. Haematological investigations revealed the presence of potent cold haemagglutinins to be the cause of her symptoms but no definite cause for the raised titres were found. She was managed conservatively by keeping her hands and feet warm and regular chlorambucil for a suspected underlying lymphoproliferative disorder. It is important to differentiate the features of Raynaud's disease from those of severe haemagglutination so that identification of any underlying disorder can be made and appropriate treatment instituted.