Idiopathic autoimmune hemolytic anemia

Alternative names
Autoimmune hemolytic anemia; Anemia - idiopathic autoimmune hemolytic

This disorder results from an abnormality of the immune system that destroys red blood cells prematurely. The cause is unknown.

Causes, incidence, and risk factors

Idiopathic autoimmune hemolytic anemia is an acquired disease that occurs when antibodies form against the person’s own red blood cells. In the idiopathic form of this disease, the cause is unknown.

There are other types of immune hemolytic anemias where the cause may result from an underlying disease or medication. Idiopathic autoimmune hemolytic anemia accounts for one-half of all immune hemolytic anemias. The onset of the disease may be quite rapid and very serious.

Risk factors are not known.


  • Fatigue  
  • Pale color  
  • Shortness of breath  
  • Rapid heartbeat  
  • Yellow skin color  
  • Dark urine  
  • Enlarged spleen

Signs and tests

  • Positive direct Coombs’ test  
  • Indirect Coombs’ test  
  • Elevated bilirubin levels  
  • Low serum haptoglobin  
  • Hemoglobin in the urine  
  • Elevated reticulocyte count  
  • Low red blood cell count and low serum hemoglobin


The first therapy tried is usuallu treatment with prednisone. If prednisone does not improve the condition, a splenectomy (removal of the spleen) may be considered.

Immunosuppressive therapy is given if the person does not respond to prednisone and splenectomy. Imuran and Cytoxan have both been used.

Blood transfusions are given with caution, if indicated for severe anemia, because of the potential that blood may not be compatible and precipitate a reaction.

Expectations (prognosis)
Adults may have chronic, relapsing disease, but in children the anemia is usually short-lived.


  • Infection (from treatment)  
  • Severe anemia

Calling your health care provider
Call your health care provider if you notice symptoms of anemia.

There is no known prevention for idiopathic autoimmune hemolytic anemia, because the cause is unknown.

Johns Hopkins patient information

Last revised: December 5, 2012
by David A. Scott, M.D.

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