Immune hemolytic anemia

Causes

Possible causes include:

• Certain chemicals, drugs, and toxins 
• Infections
• Transfusion of blood from a donor with a blood type that does not match

When antibodies form against red blood cells for no reason, it is called idiopathic autoimmune hemolytic anemia.

The antibodies may also be caused by:

• Complication of another disease
• Past blood transfusions
• Pregnancy (if the baby's blood type is different from the mother's)

Risk factors are related to the causes.

Symptoms

You may not have symptoms if the anemia is mild. If the problem develops slowly, symptoms that may occur first include:

• Feeling grumpy
• Feeling weak or tired more often than usual, or with exercise
• Headaches
• Problems concentrating or thinking

If the anemia gets worse, symptoms may include:

• Blue color to the whites of the eyes
• Brittle nails
• Light-headedness when you stand up
• Pale skin color (pallor) 
• Shortness of breath
• Sore tongue

Exams and Tests

• Absolute reticulocyte count
• Direct or indirect Coombs' test
• Hemoglobin in the urine
• LDH (level of this enzyme rises as a result of tissue damage)
• Red blood cell count (RBC), hemoglobin, and hematocrit
• Serum bilirubin levels
• Serum free hemoglobin
• Serum haptoglobin

Treatment

The first treatment tried is usually a steroid medication, such as prednisone. If steroid medications do not improve the condition, treatment with intravenous immunoglobulin (IVIG) or removal of the spleen (splenectomy) may be considered.

You may receive treatment to suppress your immune system if you do not respond to steroids and splenectomy. Medications such as azathioprine (Imuran), cyclophosphamide (Cytoxan), and rituximab (Rituxan) have been used.

Blood transfusions are given with caution, because the blood may not be compatible and it may cause more red blood cell destruction.

Outlook (Prognosis)

The disease may start quickly and be very serious, or it may remain mild and not need specific treatment.

In most people, steroids or splenectomy can control anemia. In others, treatment can usually partially control the anemia.

Possible Complications

Severe anemia rarely leads to death. Severe infection may occur as a complication of treatment with steroids, other medications that suppress the immune system, or splenectomy, because these treatments impair the body's ability to fight infection.

When to Contact a Medical Professional

Call your health care provider if you have unexplained fatigue or chest pain, or signs of infection.

Prevention

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

Alternative Names

Anemia - immune hemolytic; Autoimmune hemolytic anemia (AIHA)

References

Schwartz RS. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 163.

Powers A, Silberstein LE. Autoimmune hemolytic anemia. In: Hoffman R, Benz Ej, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 47.

Update Date: 2/7/2012

Updated by: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.