Cold agglutinin disease is a rare clinical scenario. It is usually associat
ed with infection, drug reaction, and hematologic malignancy, such as non-H
odgkin lymphoma or lymphocytic leukemia. We report a case of cold agglutini
n disease in a patient with solid-tumor uterine sarcoma. Immunological dysr
egulation has been proposed as the pathogenesis for this disease. We also s
ummarize recently reported cases of cold agglutinin disease, the underlying
conditions, and advances in the management of cold agglutinin disease.