National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Autoimmune hemolytic anemia


Other Names:
Anemia hemolytic autoimmune; Acquired autoimmune hemolytic anemia; Idiopathic autoimmune hemolytic anemia; Anemia hemolytic autoimmune; Acquired autoimmune hemolytic anemia; Idiopathic autoimmune hemolytic anemia; Familial auto-immune hemolytic anemia (subtype); Immuno-hemolytic anemia See More
Categories:
Autoimmune hemolytic anemia (AIHA) occurs when your immune system makes antibodies that attack your red blood cells. This causes a drop in the number of red blood cells, leading to hemolytic anemia.[1][2][3][4] Symptoms may include unusual weakness and fatigue with tachycardia and breathing difficulties, jaundice, dark urine and/or splenomegaly. AIHA can be primary (idiopathic) or result from an underlying disease or medication.[2] The condition may develop gradually or occur suddenly. There are two main types of autoimmune hemolytic anemia: warm antibody hemolytic anemia and cold antibody hemolytic anemia.[2][3][4] Treatment may include corticosteroids such as prednisone, splenectomy, immunosuppressive drugs and/or blood transfusions.[3]
Last updated: 3/9/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abnormal leukocyte morphology 0001881
Autoimmunity
Autoimmune disease
Autoimmune disorder
[ more ] 		0002960
Dyspnea
Trouble breathing
0002094
Fatigue
Tired
Tiredness
[ more ] 		0012378
Headache
Headaches
0002315
Hemolytic anemia 0001878
Muscle weakness
Muscular weakness
0001324
30%-79% of people have these symptoms
Immunodeficiency
Decreased immune function
0002721
Lymphoma
Cancer of lymphatic system
0002665
Pallor 0000980
5%-29% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Abnormal urinary color
Abnormal urinary colour
Abnormal urine color
[ more ] 		0012086
Arrhythmia
Abnormal heart rate
Heart rhythm disorders
Irregular heart beat
Irregular heartbeat
[ more ] 		0011675
Congestive heart failure
Cardiac failure
Cardiac failures
Heart failure
[ more ] 		0001635
Fever 0001945
Splenomegaly
Increased spleen size
0001744
Percent of people who have these symptoms is not available through HPO
Abnormality of metabolism/homeostasis
Laboratory abnormality
Metabolism abnormality
[ more ] 		0001939
Autoimmune hemolytic anemia 0001890
Autosomal recessive inheritance 0000007
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Last updated: 7/1/2020
In about half of cases, the cause of autoimmune hemolytic anemia cannot be determined (idiopathic or primary). This condition can also be caused by or occur with another disorder (secondary) or rarely, occur following the use of certain drugs (such as penicillin) or after a person has a blood and marrow stem cell transplant.[2][3][4] Secondary causes of autoimmune hemolytic anemia include:[2][4] 

Last updated: 3/9/2016
In many cases, the cause of autoimmune hemolytic anemia remains unknown.[2][3][4] Some researchers believe that there are multiple factors involved, including genetic and environmental influences (multifactorial).[2] In a very small number of cases, autoimmune hemolytic anemia appears to run in families. In these cases, it appears to follow an autosomal recessive pattern of inheritance.[5]

If you have concerns about the specific risks in your family, we encourage you to consult with a genetics professional.
Last updated: 3/9/2016
The outlook depends on the underlying cause of the disease and whether symptoms are managed appropriately and in a timely manner. Death as a result of autoimmune hemolytic anemia is rare.[2]
Last updated: 3/9/2016

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Biological differential diagnoses include other non-autoimmune causes of hemolytic anemia.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Autoimmune hemolytic anemia. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Autoimmune hemolytic anemia by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers. 
  • The National Heart, Lung, and Blood Institute (NHLBI) has information on this topic. NHLBI is part of the National Institutes of Health and supports research, training, and education for the prevention and treatment of heart, lung, and blood diseases.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Autoimmune hemolytic anemia. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • What causes autoimmune hemolytic anemia? Is it genetic? Does it usually lead to death? See answer

  • What is the outlook for people with autoimmune hemolytic anemia? See answer


  1. Gersten T. Immune hemolytic anemia. MedlinePlus. February 24, 2014; https://www.nlm.nih.gov/medlineplus/ency/article/000576.htm.
  2. Michel M. Autoimmune hemolytic anemia. Orphanet. August 2010; http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=13392.
  3. Lichtin AE. Autoimmune Hemolytic Anemia. Merck Manual. 2016; http://www.merckmanuals.com/home/blood-disorders/anemia/autoimmune-hemolytic-anemia.
  4. Hemolytic Anemia. National Heart, Lung, and Blood Institute (NHLBI). https://www.nhlbi.nih.gov/health-topics/hemolytic-anemia. Accessed 12/26/2017.
  5. ANEMIA, AUTOIMMUNE HEMOLYTIC. Online Mendelian Inheritance in Man (OMIM). November 12, 1995; http://www.omim.org/entry/205700.