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Cold agglutinin disease (CAD) is a rare autoimmune disease characterized by the presence of high concentrations of circulating cold sensitive antibodies, usually IgM and autoantibodies that are also active at temperatures below 30 °C (86 °F), [1] directed against red blood cells, causing them to agglutinate and undergo lysis. [2]
Translocation (8;22) has also been reported in association with cold agglutinin disease. Transplantation: Cold agglutinin–mediated hemolytic anemia has been described in patients after living-donor liver transplantation treated with tacrolimus and after bone marrow transplantation with cyclosporine treatments.
Primary cold agglutinin disease is idiopathic, meaning the phenomenons of agglutinations of the red blood cells and hemolysis reaction occurring outside the vessels are absent from any underlying cause. [3] Nevertheless, what is known is, those with idiopathic cold agglutinin disease are susceptible to having or developing mild clonal bone ...
Symptoms of AIHA may be due to the underlying anemia; including shortness of breath or dyspnea, fatigue, headache, muscle weakness and pallor. [10] In cold agglutinin disease (cold antibody type), agglutination and impaired passage of red blood cells through capillaries in the extremities causes acrocyanosis and Raynaud phenomenon with a rare complication of gangrene [4]
Red cell agglutination in a patient with cold agglutinin disease. In hematology, red cell agglutination or autoagglutination is a phenomenon in which red blood cells clump together, forming aggregates. It is caused by the surface of the red cells being coated with antibodies.
However, studies revealed the existence of a few cases of WAIHA that may also carry cold agglutinin antibodies that are active at the environment where the temperatures is generally equal to or warmer than 30 °C (86 °F). Such coexistence suggests a diagnosis of the mixed (warm- and cold-antibody) autoimmune hemolytic anemia abbreviated as MAIHA.
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Cold hemagglutinin disease: D59.1: 2949: Cold hemagglutinin disease (also known as cold agglutinin disease and autoimmune anemia due to cold-reactive antibodies)is an autoimmune disease characterized by the presence of high concentrations of circulating antibodies, usually IgM, directed against red blood cells. [44]