Abstract
Cold agglutinin disease (CAD) is a type of hemolytic anemia in which cold agglutinins can cause agglutination of red blood cells in cold parts of the body and hemolytic anemia. Cold agglutinin-mediated hemolytic anemia can occur in the setting of an underlying viral infection, autoimmune disorder, or lymphoid malignancy, referred to as a secondary cold agglutinin syndrome, or without one of these underlying disorders, referred to as primary CAD (also known as idiopathic CAD). We present a case of a 71-year-old female with hemolytic anemia due to primary CAD. The secondary causes of CAD, including infections, autoimmune disorders, and malignancy, were ruled out. She was successfully treated with prednisone.
Highlights
Cold agglutinin disease (CAD) is characterized by the agglutination of red blood cells (RBCs) when exposed to cold and hemolytic anemia
Cold-sensitive antibodies are usually classified into three major classes: (a) Cold agglutinins are the immunoglobulins that attach to the antigens of RBCs causing them to agglutinate and hemolyze
This hemolysis is usually extravascular, leading to anemia without significant hemoglobinuria. (b) Donath-Landsteiner antibodies attach to RBC antigens at a cold temperature similar to cold agglutinins, but, in contrast to cold agglutinins, these antibodies fix complement leading to intravascular hemolysis and cause hemoglobinemia and hemoglobinuria
Summary
Cold agglutinin disease (CAD) is characterized by the agglutination of red blood cells (RBCs) when exposed to cold (usually in the extremities) and hemolytic anemia. Cold-sensitive antibodies are usually classified into three major classes: (a) Cold agglutinins are the immunoglobulins that attach to the antigens of RBCs causing them to agglutinate and hemolyze. This hemolysis is usually extravascular, leading to anemia without significant hemoglobinuria. (c) Cryoglobulins are the third type of cold-sensitive antibodies that form immune complexes in the cold temperature and cause systemic vasculitis These antibodies usually do not react with RBCs. Cold agglutinin-associated autoimmune hemolytic anemia (AIHA) can be primary (idiopathic) or secondary (due to viral infections, lymphoproliferative disorder, or autoimmune disorder) [1]. The patient was referred to the emergency department by her primary care physician due to low hemoglobin of 5.1 g/dL on routine blood work. Her prednisone dose has been gradually tapered down to 5 mg/day with stable hemoglobin counts
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