Cold autoimmune hemolytic anemia

Abstract

Cold agglutinins are antibodies that recognize antigens on erythrocytes at temperatures below normal body temperature. Antibodies are of IgM nature and bind to “I” or “i” antigens on red blood cells, causing agglutination in red blood cells. This situation results in anemia by creating extravascular hemolysis. If there is no underlying disease, it is called primary or idiopathic cold agglutinin disease (CAD), and if there is, it is called secondary cold agglutinin syndrome (CAS). Primary CAD is an extremely rare disease, with an incidence and prevalence of 1 per million and 16 per million, respectively. It is seen twice more in women than in men, and the median age of diagnosis is 67 (between 30-92 years). The etiology of CAS includes infections, autoimmune and lymphoproliferative diseases. There are cold-related symptoms and symptoms of anemia in the clinic. In treatment, it is necessary to avoid cold in order to reduce cold-induced symptoms and hemolysis. Currently, the most effective treatment for reducing antibody production is rituximab. It can be given alone or in combination with bendamustine, interferon alfa, fludarabine and prednisolone. bortezomib is used when rituximab is ineffective or contraindicated. Plasmapheresis or intravenous immunoglobulin can be given when there is critical hemolysis or when the effectiveness of immunosuppressive therapy may start late. Treatment in CAS is the treatment of the underlying disease.