What is the etiology of autoimmune hemolytic anemia (AIHA)?

Updated: May 27, 2021
  • Author: Srikanth Nagalla, MD, MS, FACP; Chief Editor: Emmanuel C Besa, MD  more...
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Autoimmune hemolytic anemia (AIHA) can be due to warm or cold autoantibody types and, rarely, mixed types. [15, 22, 23] Most warm autoantibodies belong to the immunoglobulin IgG class. These antibodies can be detected by a direct Coombs test, which also is known as a direct antiglobulin test (DAT). AIHA may occur after allogeneic hematopoietic stem cell transplantation. The 3-year cumulative incidence in this population has been reported at 4.44%. [25]

AIHA is rare in children and has a range of causes. Autoimmune hemolysis can be primary or secondary to conditions such as infections (viral, bacterial, and atypical), systemic lupus erythematosus (SLE), autoimmune hepatitis (AIH), and  H1N1 influenza. H1N1 influenza–associated AIHA in children may respond to treatment with oseltamivir and intravenous immunoglobulin. [8]

Fetal splenomegaly and associated hepatomegaly could be due to hemolysis, but infections are the most likely cause.  Congestive heart failure and metabolic disorders should be considered. Rarely, leukemia, lymphoma, and histiocytosis are associated with splenomegaly. [26]

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