[Mixed warm and cold antibody type autoimmune hemolytic anemia associated with systemic lupus erythematosus]

A 56-year-old woman developed mixed warm and cold antibody type autoimmune hemolytic anemia (mixed AIHA) associated with systemic lupus erythematosus. The patient was admitted to our hospital for acrocyanosis and shortness of breath. High fever and jaundice were observed. Urinalysis revealed protein and hemoglobin, and the sediment contained granular and hyaline casts. Her erythrocytes agglutinated markedly at room temperature. Her hemoglobin was 5.6 g/dl and reticulocyts were 19.3%. Total bilirubin, GOT and LDH were elevated, while haptoglobin and complements were abnormally reduced. Polyclonal increase of immunoglobulin, ANA and anti-Sm antibody were detected. The direct antiglobulin test was positive; IgG1, IgG3 and C3d were detected on the red cell surface. The cold agglutinin (CA) titer was 4096, showing anti-I blood group specificity, and was still active at 30 degrees C. Upon administration of prednisolone gradual increase of hemoglobin and decrease of reticulocytes were observed, indicating the healing of hemolysis. CA disappeared but the direct antiglobulin test remained positive. Mixed AIHA has been defined as the presence of both warm and cold antibodies. In addition, the presence of symptoms of cold agglutinin disease, or low-titer and high thermal amplitude CA might be necessary for the diagnosis of mixed AIHA.