What is Autoimmune Hemolytic Anemia (AIHA)?

If you or a loved one is affected by wAIHA and would like to learn about the latest research, connect with one of our patient supporters here.

Autoimmune hemolytic anemia (AIHA) is a rare form of anemia caused by a malfunction of the immune system.

Anemia happens when the body does not have enough red blood cells. When someone has AIHA, their immune system creates proteins (called antibodies) that attack and destroy the body's own red blood cells. 

AIHA is a complicated condition that affects people in different ways. Some of the most common symptoms are fatigue, weakness, dizziness, and shortness of breath.

It is not always possible to completely cure AIHA, but the condition can be managed. Treatment can help stop the destruction of red blood cells and improve symptoms for most people.

The Role of Red Blood Cells in AIHA

Red blood cells are responsible for transporting oxygen to all the cells of the body. A lack of red blood cells to carry oxygen can leave a person tired, short of breath, and looking pale. The medical term for a lack of red blood cells is anemia.

Red blood cells contain an important protein called hemoglobin. Hemoglobin contains iron, which binds easily to oxygen. Hemoglobin also gives blood its red color.

Hemoglobin in the red blood cells attaches to oxygen molecules at the point where the lungs come into contact with the bloodstream. After delivering oxygen to the body's cells, the red blood cells pick up carbon dioxide and carry it back to the lungs to be exhaled.

Red blood cells play a role in helping the immune system defend against invading bacteria or viruses. Red blood cells can release substances that cause the blood vessels to dilate when necessary.

The Types of  Autoimmune Hemolytic Anemia (AIHA)

AIHA is classified in a number of different ways. Figuring out what kind of AIHA a person has helps doctors determine the best treatment for each case.

In general, AIHA is categorized as either primary or secondary. AIHA is also classified as warm-antibody AIHA or cold-antibody AIHA according to the optimal temperature at which the antibodies destroy red blood cells

Primary AIHA

Primary AIHA is AIHA that has no apparent cause. About half of all cases of AIHA are primary. This type of AIHA is more likely than secondary AIHA to be asymptomatic (causing no symptoms). Primary AIHA is also more likely than secondary to be life-threatening. Another name for primary AIHA is idiopathic AIHA. Idiopathic is a medical term meaning “of unknown cause.”

Secondary AIHA

Secondary AIHA, also called acquired AIHA, is AIHA caused by an underlying health condition. Health conditions commonly associated with secondary AIHA include autoimmune disorders (for example, lupus or rheumatoid arthritis) and blood cancers (such as lymphocytic leukemia or lymphoma). Secondary AIHA may also be caused by infection or a reaction to a drug.

What is the difference between warm and cold autoimmune anemia?

Immunoglobulin G (IgG) is the antibody associated with most cases of warm-antibody AIHA. Cold-antibody AIHA is most likely to be caused by the immunoglobulin M (IgM) antibody.

Warm-Antibody Autoimmune Anemia

  • Red blood cells are destroyed at normal body temperature 

  • 70% to 80% of AIHA cases

  • Affects men and women equally

  • The antibody is almost always IgG

  • 50% of cases are primary, 50% are secondary

  • The Secondary kind of Warm-Antibody anemia is likely caused by an autoimmune or lymphoproliferative disorder

Cold-Antibody Autoimmune Anemia

  • Red blood cells are destroyed at temperatures below normal body temperature

  • Up to 20% of AIHA cases

  • Older women more likely to be affected

  • The antibody is usually IgM

  • Most often associated with secondary causes

  • The secondary cause is most likely infection or a lymphoproliferative disorder

 

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References:

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Barcellini W. New insights in the pathogenesis of autoimmune hemolytic anemia. Transfus Med Hemother. 2015;42:287–93.

Barros MMO, Blajchman MA, Bordin JO. Warm autoimmune hemolytic anemia: recent progress in understanding the immunobiology and the treatment. Transfus Med Rev. 2010: 24(3): 195–210.

Barros MMO, Langhi DM, Bordin JO. Autoimmune hemolytic anemia: transfusion challenges and solutions. Int J Clin Transfus Med. 2017; 5: 9–18.

Chang A, Chaturvedi S, McCrae K. Thirteen year retrospective analysis of adult patients with autoimmune hemolytic anemia at the Cleveland Clinic: diagnosis and prevalence of associated disorders. Blood. 2013; 122:955 1–5.

Hill QA, Stamps R. Massey E, et al. The diagnosis and management of primary autoimmune haemolytic anaemia. BJH. 2017; 176: 395–411.

Michel M. Classification and therapeutic approaches in autoimmune hemolytic anemia: an update. Expert Rev Hematol. 2011; 4(6): 607–18.

Seve P, Phillipe P, Dufour J-F, et al. Autoimmune hemolytic anemia: classification and therapeutic approaches. Expert Rev Hematol. 2008; 1(2): 189-204.

Zanella A, Barcellini W. Treatment of autoimmune hemolytic anemias. Haematologica. 2104; 99(10): 1547–54.


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