A Novel Approach for Treatment of Cold Agglutinin Syndrome-Related Severe Hemolysis

Abstract

Intravascular hemolysis related to cold agglutinin syndrome results from the activation of the classical complement pathway by red blood cell (RBC) surface I/i antigen bound autoantibodies. Despite built-in mechanisms that limit continued downstream complement activation, some patients may develop life-threatening intravascular hemolysis due to the formation of membrane attack complexes. We present a case of severe intravascular hemolysis due to cold agglutinin syndrome that was treated successfully using proximal complement inhibition with commercial C1 esterase inhibitor. The role of complement inhibition in controlling the intravascular hemolysis and resolving the immune dysfunction that leads to the syndrome is discussed. J Hematol. 2016;5(1):30-33 doi: http://dx.doi.org/10.14740/jh242w