Autoimmune Hemolytic Anemia: Everything You Need to Know

Table of ContentsView AllTable of ContentsWhat Is Hemolysis?What Is AIHA?Types and Risk FactorsOther CausesSymptomsDiagnosisTreatmentTreatment for Warm vs. Cold AIHAAIHA in ChildrenPreventionCoping

Table of ContentsView All

View All

Table of Contents

What Is Hemolysis?

What Is AIHA?

Types and Risk Factors

Other Causes

Symptoms

Diagnosis

Treatment

Treatment for Warm vs. Cold AIHA

AIHA in Children

Prevention

Coping

Autoimmune hemolytic anemia (AIHA) is a rareautoimmune disorderthat causes the body to mistake its ownred blood cells(RBCs) for foreign substances. Then, your body’s immune system creates autoantibodies to destroy red blood cells before they can be replaced, leading toanemia(low red blood cell count).

Without the proper amount of red blood cells, you may experience symptoms like fatigue, shortness of breath, dizziness, and weakness. If untreated, autoimmune hemolytic anemia sometimes leads to severe complications likeheart failure.

In this article, we’ll review everything you need to know about autoimmune hemolytic anemia, including types, risk factors, causes, symptoms, treatment options, and more.

Andrew Brookes / Getty Images

What Is Autoimmune Hemolytic Anemia (AIHA)?

Autoimmune hemolytic anemia is an autoimmune condition, meaning that it causes the body to mistakenly attack its own healthy tissues. In the case of AIHA, the body’s immune system attacks and destroys its own red blood cells.

Due to their low red blood cell count, people with AIHA may feel dizzy, light-headed, or weak. They also sometimes experience problems with their spleen, liver, or both.

AIHA is manageable when identified, diagnosed, and treated early.However, if left untreated, it can lead to serious and even fatal complications. The estimated one-year survival rate for people with AIHA is 79.5%.Health complications from unresolved AIHA may include:

Autoimmune hemolytic anemia is a group of closely related disorders There are different types of autoimmune hemolytic anemia, including warm, cold, primary, and secondary AIHA.

Warm AIHA

Potential complications of warm AIHA include an enlarged spleen,deep vein thrombosis(DVT), andpulmonary embolism.

Cold AIHA

Cold AIHA is less common than warm AIHA and typically affects people between 40 and 80 years old. Some people with cold AIHA develop problems with circulation, such asRaynaud’s syndrome.

Primary or Secondary AIHA

About 50% of people with autoimmune hemolytic anemia have primary AIHA, which means that it’s not due to any apparent comorbid condition or underlying cause. This is also known as idiopathic autoimmune hemolytic anemia.

Another 50% have secondary AIHA, which means that it develops because of an underlying related condition. Possible causes of secondary AIHA include malignancy (cancer),viruses, certain medications, and autoimmune disorders.

Other AIHA Causes

Hemolytic Anemia Symptoms

Because red blood cells carry oxygen throughout your body, a low red blood cell count can lead to a number of symptoms. Symptoms of hemolytic anemia include:

AIHA Diagnosis

To diagnose you with AIHA, your healthcare provider may perform a physical exam, bone marrow test, and/or blood tests.Blood tests for AIHA include:

Autoimmune Hemolytic Anemia Treatment

Autoimmune hemolytic anemia can be treated with medication, surgery, and/or blood transfusions.

Medication

Steroid therapy, particularly with prednisone, is the first line of treatment for most cases of AIHA. An estimated 80% of people with AIHA who are treated with glucocorticoids will see their symptoms resolve within up to three weeks. If your symptoms persist, you may be prescribed an immunosuppressant instead.

Rituximab, a type of monoclonal antibody, also treats autoimmune hemolytic anemia. It can be prescribed either alone or alongside prednisone to treat AIHA symptoms.

Surgery (Splenectomy)

If steroids and other medications don’t work, some people with warm AIHA may need to undergo asplenectomy. Splenectomy is the surgical removal of the spleen. An estimated 40%–90% of people with warm AIHA respond favorably after getting a splenectomy. However, the surgery has risks, such as blood clots and infections.

Blood Transfusion

If you have severe AIHA symptoms, your healthcare provider may recommend ablood transfusionto replace your red blood cells. Some people with AIHA will need to get blood transfusions on an ongoing basis if they continue to relapse.

Warm AIHA is typically treated with steroids and/or rituximab. Meanwhile, cold AIHA doesn’t always require treatment. Mild cases of cold AIHA may resolve on their own. People with cold AIHA should stay out of the cold, as their red blood cells will be destroyed more quickly in cooler climates. In more severe cases, people withcold agglutinin disease(CAD) may need blood transfusions and/or rituximab. Splenectomies are typically not recommended for people with CAD.

Very few babies and children develop AIHA. Among those with childhood autoimmune hemolytic anemia, about 37% have primary AIHA and about 53% have secondary AIHA. Cold AIHA is especially rare in younger patients.

Children with secondary AIHA often develop the condition due to a comorbid (co-occurring) immune disorder, such as a platelet disorder calledimmune thrombocytopenia(ITP). Treatment for pediatric AIHA is similar to the treatment options for adults, with steroids as the first-line therapy.

AIHA Prevention

Also, talk to a healthcare provider right away if you have any symptoms of AIHA. Early treatment can resolve your condition and prevent complications.

Lifestyle Management for AIHA

If you have AIHA, it’s important to visit a healthcare provider regularly so they can monitor any conditions you have and prevent them from getting worse.

If you have ongoing symptoms or are taking steroids, your healthcare provider may also recommend that you take the following supplements:

Finally, if you have cold AIHA, avoiding cold environments and staying warm as much as possible is important.

Summary

Autoimmune hemolytic anemia is a condition that causes your body to break down red blood cells too quickly to be replaced. Symptoms include dizziness, weakness, rapid heart rate, headaches, jaundice, and an enlarged spleen or liver.

AIHA can usually be effectively treated with medication, such as steroids. Surgery or blood transfusions may be necessary if symptoms don’t improve.

16 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Michalak SS, Olewicz-Gawlik A, Rupa-Matysek J, Wolny-Rokicka E, Nowakowska E, Gil L.Autoimmune hemolytic anemia: Current knowledge and perspectives.Immun Ageing. 2020;17(1):38. doi:10.1186/s12979-020-00208-7National Heart, Lung, and Blood Institute.Hemolytic anemia.MedlinePlus.Hemolysis.MedlinePlus.Hemolytic anemia.National Organization for Rare Disorders.Warm autoimmune hemolytic anemia.Hansen DL, Möller S, Frederiksen H.Survival in autoimmune hemolytic anemia remains poor, results from a nationwide cohort with 37 years of follow-up.Eur J Haematol. 2022;109(1):10-20. doi:10.1111/ejh.13764National Organization for Rare Disorders.Cold agglutinin disease.Hill A, Hill QA.Autoimmune hemolytic anemia.Hematology Am Soc Hematol Educ Program. 2018;2018(1):382-389. doi:10.1182/asheducation-2018.1.382MedlinePlus.CBC blood test.MedlinePlus.Coombs test.MedlinePlus.Reticulocyte test.MedlinePlus.Bilirubin blood test.MedlinePlus.Haptoglobin blood test.MedlinePlus.Febrile/cold agglutinins.Zanella A, Barcellini W.Treatment of autoimmune hemolytic anemias.Haematologica. 2014;99(10):1547-54. doi:10.3324/haematol.2014.114561Voulgaridou A, Kalfa TA.Autoimmune hemolytic anemia in the pediatric setting.J Clin Med. 2021;10(2):216. doi:10.3390/jcm10020216

16 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Michalak SS, Olewicz-Gawlik A, Rupa-Matysek J, Wolny-Rokicka E, Nowakowska E, Gil L.Autoimmune hemolytic anemia: Current knowledge and perspectives.Immun Ageing. 2020;17(1):38. doi:10.1186/s12979-020-00208-7National Heart, Lung, and Blood Institute.Hemolytic anemia.MedlinePlus.Hemolysis.MedlinePlus.Hemolytic anemia.National Organization for Rare Disorders.Warm autoimmune hemolytic anemia.Hansen DL, Möller S, Frederiksen H.Survival in autoimmune hemolytic anemia remains poor, results from a nationwide cohort with 37 years of follow-up.Eur J Haematol. 2022;109(1):10-20. doi:10.1111/ejh.13764National Organization for Rare Disorders.Cold agglutinin disease.Hill A, Hill QA.Autoimmune hemolytic anemia.Hematology Am Soc Hematol Educ Program. 2018;2018(1):382-389. doi:10.1182/asheducation-2018.1.382MedlinePlus.CBC blood test.MedlinePlus.Coombs test.MedlinePlus.Reticulocyte test.MedlinePlus.Bilirubin blood test.MedlinePlus.Haptoglobin blood test.MedlinePlus.Febrile/cold agglutinins.Zanella A, Barcellini W.Treatment of autoimmune hemolytic anemias.Haematologica. 2014;99(10):1547-54. doi:10.3324/haematol.2014.114561Voulgaridou A, Kalfa TA.Autoimmune hemolytic anemia in the pediatric setting.J Clin Med. 2021;10(2):216. doi:10.3390/jcm10020216

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

Michalak SS, Olewicz-Gawlik A, Rupa-Matysek J, Wolny-Rokicka E, Nowakowska E, Gil L.Autoimmune hemolytic anemia: Current knowledge and perspectives.Immun Ageing. 2020;17(1):38. doi:10.1186/s12979-020-00208-7National Heart, Lung, and Blood Institute.Hemolytic anemia.MedlinePlus.Hemolysis.MedlinePlus.Hemolytic anemia.National Organization for Rare Disorders.Warm autoimmune hemolytic anemia.Hansen DL, Möller S, Frederiksen H.Survival in autoimmune hemolytic anemia remains poor, results from a nationwide cohort with 37 years of follow-up.Eur J Haematol. 2022;109(1):10-20. doi:10.1111/ejh.13764National Organization for Rare Disorders.Cold agglutinin disease.Hill A, Hill QA.Autoimmune hemolytic anemia.Hematology Am Soc Hematol Educ Program. 2018;2018(1):382-389. doi:10.1182/asheducation-2018.1.382MedlinePlus.CBC blood test.MedlinePlus.Coombs test.MedlinePlus.Reticulocyte test.MedlinePlus.Bilirubin blood test.MedlinePlus.Haptoglobin blood test.MedlinePlus.Febrile/cold agglutinins.Zanella A, Barcellini W.Treatment of autoimmune hemolytic anemias.Haematologica. 2014;99(10):1547-54. doi:10.3324/haematol.2014.114561Voulgaridou A, Kalfa TA.Autoimmune hemolytic anemia in the pediatric setting.J Clin Med. 2021;10(2):216. doi:10.3390/jcm10020216

Michalak SS, Olewicz-Gawlik A, Rupa-Matysek J, Wolny-Rokicka E, Nowakowska E, Gil L.Autoimmune hemolytic anemia: Current knowledge and perspectives.Immun Ageing. 2020;17(1):38. doi:10.1186/s12979-020-00208-7

National Heart, Lung, and Blood Institute.Hemolytic anemia.

MedlinePlus.Hemolysis.

MedlinePlus.Hemolytic anemia.

National Organization for Rare Disorders.Warm autoimmune hemolytic anemia.

Hansen DL, Möller S, Frederiksen H.Survival in autoimmune hemolytic anemia remains poor, results from a nationwide cohort with 37 years of follow-up.Eur J Haematol. 2022;109(1):10-20. doi:10.1111/ejh.13764

National Organization for Rare Disorders.Cold agglutinin disease.

Hill A, Hill QA.Autoimmune hemolytic anemia.Hematology Am Soc Hematol Educ Program. 2018;2018(1):382-389. doi:10.1182/asheducation-2018.1.382

MedlinePlus.CBC blood test.

MedlinePlus.Coombs test.

MedlinePlus.Reticulocyte test.

MedlinePlus.Bilirubin blood test.

MedlinePlus.Haptoglobin blood test.

MedlinePlus.Febrile/cold agglutinins.

Zanella A, Barcellini W.Treatment of autoimmune hemolytic anemias.Haematologica. 2014;99(10):1547-54. doi:10.3324/haematol.2014.114561

Voulgaridou A, Kalfa TA.Autoimmune hemolytic anemia in the pediatric setting.J Clin Med. 2021;10(2):216. doi:10.3390/jcm10020216

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