Dry gangrene in cold agglutinin syndrome.

Abstract

A 78-year-old male patient was admitted to hospital because of gangrene in his fingers for a month. On physical examination (Fig. 1), he showed dry gangrene of the fingers and toes with normal pulsation of the bilateral radial artery, ulnar artery and dorsal foot artery. His blood had spontaneously agglutinated during the blood draw. Laboratory evaluation revealed a haemoglobin level of 64 g/l, an indirect bilirubin level of 17.2 μmol/l and positive urobilinogen. In addition, a positive direct anti-human globulin test and indirect anti-human globulin test, and a positive irregular anti-erythrocyte antibody screening test (antibodies outside of the ABO system) were also found. Moreover, the test for cold agglutinins was positive at a 1:512 dilution at 4°C. As a result, he was diagnosed with cold agglutinin syndrome. Cold agglutinin syndrome is an autoimmune haemolytic anaemia without other underlying diseases [1, 2]. The main clinical manifestations include anaemia, cold-induced acrocyanosis, RP and even gangrene. It may be exacerbated by the cold weather in winter in Southwest China. It is essential to keep warm and avoid cold environments. In addition, an in-line blood warmer, plasma exchange and clonally directed therapies are also effective [2].