Idiopathic autoimmune hemolytic anemia

Idiopathic autoimmune hemolytic anemia

Definition:

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

Alternative Names:

Autoimmune hemolytic anemia; Anemia - idiopathic autoimmune hemolytic

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.

Symptoms:

Signs and tests:

positive Coombs' test, direct
Coombs' test, indirect
elevated bilirubin levels
low serum haptoglobin
hemoglobin in the urine
elevated reticulocyte count
low red blood cell count and serum 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 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.

Complications:

infection (from treatment)
severe anemia

Calling your health care provider:

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

Prevention:

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

Review Date: 10/19/2001
Reviewed By: Rebecca Elstrom, M.D., Division of Hematology-Oncology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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