Hemolytic anemia

haemolytic anaemia

Basic Information

What is Hemolytic anemia?

The hemolytic anemias are a category of diseases seen when red blood cells have a shortened life span and are broken down faster than they can be produced. This results in an anemia, a reduction in the total number of red blood cells.

Hemolytic anemia may be due to two general causes: an inherent defect in the red blood cell, as seen in sickle cell anemia, hereditary spherocytosis, and thalassemia, or a disorder outside of the red blood cell. Disorders outside the cell that may cause hemolytic anemia include the person's immune system inappropriately attacking his/her own red blood cells (autoimmune hemolytic anemia), an appropriate immune attack of mismatched red blood cells in a transfusion reaction, an adverse effect of a drug taken for another disease, or excessive trapping of blood cells in the spleen (hypersplenism).

Hemolytic anemia signs and symptoms

  • Fatigue.
  • Shortness of breath.
  • Irregular heartbeat.
  • Jaundice (yellow skin and eyes, dark urine).
  • Enlarged spleen.

Causes

  • Often the cause is unknown.
  • Inherited disorder, such as hereditary spherocytosis, glucose-6-phosphate dehydrogenase (G6PD) deficiency, sickle-cell anemia or thalassemia.
  • Favism (an acute hemolytic anemia caused by the ingestion of the fava bean) is common in certain individuals, primarily of Greek descent, due to a hereditary biochemical lesion.
  • Antibodies produced by the body to fight infections, which for unknown reason attack red blood cells. This response is sometimes triggered by blood transfusions.
  • Use of medications, including non-prescription drugs, that damage red blood cells.

How is it diagnosed?

  • History will vary depending on how fast the hemolytic anemia develops. An individual with slow development may only report some yellowing of the skin and eyes (jaundice). Individuals with a faster onset of hemolytic anemia may have fatigue and shortness of breath. With an acute onset, there may be fever, chills, headache, and pain in the back, abdomen, or extremities. The urine may become red or dark. There is no history of abnormal blood loss with hemolytic anemia.
  • Physical exam findings include pallor, a fast heart rate, and heart murmur. Mild jaundice is usually present. The spleen and liver may be enlarged. Signs of heart failure can be seen with severe anemia.
  • Tests: A complete blood count (CBC) demonstrates the degree of anemia, and a blood smear will show an increased number of immature red blood cells (reticulocytes). Serum bilirubin will be elevated. The direct Coombs test will be positive in immune hemolytic anemias.

Risk increases with

  • Family history of hemolytic anemia.
  • Use of any medication.

Preventive measures

  • Don't take any medicine that has previously triggered hemolytic anemia.
  • Seek genetic counseling before having children if you have a family history of hemolytic anemia (inherited forms).

Expected outcomes

  • If hemolytic anemia is acquired, it can usually be cured when the cause, such as a drug, is removed. Sometimes it is necessary to remove the spleen surgically.
  • If secondary to an underlying disorder, the prognosis is determined by the course of the primary disease.
  • If hemolytic anemia is inherited, it is currently considered incurable. However, symptoms can be relieved or controlled.
  • Scientific research into causes and treatment continues, so there is hope for increasingly effective treatment and cure.

Possible complications

  • Excessive spleen enlargement, which increases destruction of red blood cells.
  • Pain, shock and serious illness caused by hemolysis (red-blood-cell destruction).
  • Gallstones.

Hemolytic anemia treatment

General measures

  • Diagnostic tests will include laboratory blood studies.
  • Treatment is individualized depending on the specific hemolytic problem.
  • Some types may be preventable by avoidance of the drugs or foods that precipitate hemolysis.
  • Surgical removal of the spleen may be recommended.

Medications

  • Immunosuppressive (e.g., prednisone) drugs to control the antibody response are usually prescribed.
  • Medication to reduce pain. For minor discomfort, you may use non-prescription drugs such as acetaminophen.
Information Brand Generic Label Rating
Danazol capsules Danocrine Danazol
Imuran Azathioprine
Cytoxan Cyclophosphamide

Activity

  • After treatment, resume normal activities as soon as possible.
  • Avoid cold temperatures.

Diet

No special diet.

Alternatives

Thrombotic thrombocytopenic purpura (TTP) and disseminated intravascular coagulopathy can initially resemble hemolytic anemia.

Appropriate specialists

Hematologist.

Notify your physician if

  • You or a family member has symptoms of hemolytic anemia.
  • The following occur during treatment:
    • Fever.
    • Cough.
    • Sore throat.
    • Swollen joints.
    • Muscle aches.
    • Bloody urine.
    • Signs of infection in any part of the body (redness, pain, swelling, fever).
  • New, unexplained symptoms develop. Drugs used in treatment may produce side effects.

Last updated 27 May 2012


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