Autoimmune hemolytic anemias and paroxysmal nocturnal hemoglobinuria

Andrea M. McGonigle, Paul M. Ness, Karen E. King

Research output: Chapter in Book/Report/Conference proceedingChapter


The hallmark of both autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria is shortened red cell survival; however the mechanisms leading to this common feature differ significantly. In autoimmune hemolytic anemia, autoantibodies directed against the patient’s own red cells lead to accelerated red cell destruction. The autoimmune hemolytic anemias are classified based on the characteristics of the responsible autoantibodies and their clinical settings. Warm autoimmune hemolytic anemia is due to an IgG autoantibody directed against red cell antigens optimally reactive at 37°C. Cold agglutinin disease is caused by an IgM which is optimally reactive at 0°C. Paroxysmal cold hemoglobinuria is caused by a biphasic hemolysin, the Donath-Landsteiner antibody, which is an IgG antibody that binds red cells at lower temperatures, activates complement and causes hemolysis at warmer temperatures. In drug-induced immune hemolytic anemia, specific drugs induce a hemolytic anemia which is clinically indistinguishable from warm autoimmune hemolytic anemia. In contrast to the autoimmune hemolytic anemias, paroxysmal nocturnal hemoglobinuria occurs in the context of a somatic gene mutation and some component of bone marrow dysfunction. This somatic mutation, that results in decreased glycosylphosphotidylinositol anchored proteins, ultimately leads to increased sensitivity to complement with effects of intravascular hemolysis and increased risk of thrombosis.

Original languageEnglish (US)
Title of host publicationRossi’s Principles of Transfusion Medicine
Number of pages15
ISBN (Electronic)9781119013020
ISBN (Print)9781119012993
StatePublished - Apr 15 2016


  • Antiglobulin test
  • Autoantibody
  • Autoimmune
  • Biphasic hemolysin
  • Cold agglutinin disease
  • Donath-landsteiner antibody
  • Drug-induced immune hemolytic anemia
  • Hemolysis/hemolytic
  • Paroxysmal cold hemoglobinuria
  • Paroxysmal nocturnal hemoglobinuria

ASJC Scopus subject areas

  • General Medicine


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