Immune hemolytic anemia

Alternative names
Anemia - immune hemolytic

Definition
Immune hemolytic anemia is a disorder characterized by anemia due to premature destruction of red blood cells by the immune system.

Causes, incidence, and risk factors

Immune hemolytic anemia occurs when antibodies form against the body’s own red blood cells. The antibodies may be acquired by blood transfusion, pregnancy (if the baby’s blood type is different from the mother’s), as a complication of another disease, or from a reaction to medications.

If the cause of antibody formation is disease or medication, it is referred to as secondary immune hemolytic anemia. The antibodies will destroy the blood cells because they are recognized as foreign substances within the body.

The cause may also be unknown, as in idiopathic autoimmune hemolytic anemia, which accounts for one-half of all immune hemolytic anemias. The onset of the disease may be quite rapid and very serious, or it may remain mild and not require specific therapy. Risk factors are related to the causes.

Symptoms

     
  • Fatigue  
  • Pale or yellow skin color  
  • Shortness of breath  
  • Rapid heart rate  
  • Dark urine  
  • Enlarged spleen

Signs and tests

     
  • Positive direct Coombs’ test or indirect Coombs’ test  
  • Elevated bilirubin levels  
  • Elevated LDH  
  • Low serum haptoglobin  
  • Hemoglobin in the urine  
  • Elevated absolute reticulocyte count  
  • Low red blood cell count and hemoglobin

Treatment
Treatment with prednisone is the first therapy that is tried. If prednisone does not improve the condition, a splenectomy (removal of the spleen) may be considered. Immunosuppressive therapy will be given if the person does not respond to prednisone and splenectomy.

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

Expectations (prognosis)
In most people, steroids or splenectomy control anemia. In others, partial control of the anemia is usually achieved.

Complications
Death rarely occurs from severe anemia. Overwhelming infection may occur as a complication of therapy with steroids or splenectomy, since they reduce the body’s ability to fight infection.

Calling your health care provider
Call your health care provider if unexplained fatigue or chest pain occurs, or if signs of infection are present.

Prevention
Screening for antibodies in donated blood and in the recipient may prevent hemolytic anemia related to blood transfusions.

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

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