What are the differences between primary and secondary cold agglutinin disease?

Updated: Aug 28, 2018
  • Author: Salman Abdullah Aljubran, MD; Chief Editor: Michael A Kaliner, MD  more...
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Primary cold agglutinin disease is usually associated with monoclonal cold-reacting autoantibodies. Primary cold agglutinin disease is chronic and occurs after the fifth decade of life, with a peak incidence in the seventh and eighth decades. (See Epidemiology.)

Secondary cold agglutinin disease may be associated with either monoclonal or polyclonal cold-reacting autoantibodies. It predominantly is caused by infection and lymphoproliferative disorders. Monoclonal secondary disease is usually chronic, occurring in adults. Polyclonal secondary cold agglutinin disease, which occurs in children and young adults, is usually transient. (See Etiology and Prognosis.)

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