Skip to content
How It Works
Explore
Resources Ask a Question
Who It's For
Patients
Decode results, prep for appointments, weigh options.
Caregivers & advocates
Carry the research load for someone you love.
Providers & clinicians
Resources for patients in your practice.
Log In Ask a Question
PubMed This is a summary of 73 peer-reviewed journal articles Updated
Hematology

Understanding Autoimmune Hemolytic Anemia: A Patient's Guide

At a Glance

Autoimmune Hemolytic Anemia (AIHA) is a rare but highly treatable blood disorder where the immune system mistakenly attacks healthy red blood cells. Symptoms include severe fatigue, jaundice, and dark urine. Diagnosis typically involves a Coombs test ordered by a specialist called a hematologist.

It is completely normal to feel overwhelmed when you first hear the words Autoimmune Hemolytic Anemia (AIHA). This is a rare condition, and the symptoms can feel both sudden and exhausting. However, while AIHA is a serious diagnosis, it is a well-understood and highly manageable condition [1][2]. Understanding why your body is reacting this way is the first step toward taking control of your health.

What is AIHA?

In a healthy body, red blood cells (RBCs) carry oxygen to all your tissues. Usually, these cells live for about 120 days before the body replaces them. In AIHA, your immune system—which is supposed to protect you—mistakenly creates autoantibodies (proteins that attack your own tissues) that target your red blood cells [3][4].

These antibodies “tag” your red blood cells for destruction, causing them to be broken down much faster than your bone marrow can replace them [4][5]. This process of cell destruction is called hemolysis [1].

Why This Might Be New to Your Local Doctor

AIHA is rare. It affects approximately 1 to 3 people out of every 100,000 each year [6][7]. Because of this rarity, many primary care physicians may only see one or two cases in their entire careers.

Symptoms of AIHA—like extreme tiredness or feeling winded—often look like more common problems like the flu, stress, or general iron deficiency [8][9]. This can lead to a “diagnostic pitfall” where the true cause is missed initially [10]. This is why it is essential to work with a hematologist, a specialist who focuses specifically on blood disorders and has the expertise to manage this rare condition [11][12].

Recognizing the Symptoms

When your red blood cell count drops quickly, your body sends out clear distress signals. You may experience:

  • Extreme Fatigue and Lethargy: A level of tiredness that sleep doesn’t fix, caused by a lack of oxygen reaching your muscles and brain [3][1].
  • Jaundice: A yellowing of the skin or the whites of the eyes. This happens because the rapid breakdown of red blood cells releases a yellow pigment called bilirubin [1][13].
  • Dark Urine: Often described as looking like tea or cola. This occurs when the remnants of destroyed red blood cells are filtered out by your kidneys [3][13].
  • Shortness of Breath: Feeling winded even during simple activities like walking or climbing stairs [8].

Primary vs. Secondary AIHA

Doctors categorize AIHA based on whether it started on its own or was triggered by something else.

  • Primary AIHA: This is “idiopathic,” meaning it occurs on its own without an obvious outside cause [14].
  • Secondary AIHA: This is triggered by an underlying condition. Common triggers include:
    • Autoimmune diseases such as Systemic Lupus Erythematosus (SLE or Lupus) [15][16].
    • Blood cancers like Chronic Lymphocytic Leukemia (CLL) [17][18].
    • Infections, including viral infections like SARS-CoV-2 (COVID-19) or bacterial infections like Mycoplasma pneumonia [15][19].
    • Drug-Induced Immune Hemolytic Anemia (DIIHA): Sometimes, certain medications (like specific antibiotics) can trigger the immune system to attack red blood cells. Your doctor will likely review your medication list to rule this out [11].

Finding the Path Forward

The road to recovery begins with a clear diagnosis, usually confirmed by a Direct Antiglobulin Test (DAT), also known as the Coombs test [20][5]. This test identifies the specific antibodies on your red blood cells.

While the initial phase of AIHA can be intense, modern treatments are highly effective at stabilizing blood counts and helping patients return to their normal lives [21][22][23]. AIHA is a chronic journey, but you do not have to walk it alone.

Guide Navigation

To help you navigate your diagnosis and treatment, we have prepared the following detailed guides:

01

The Types of AIHA: Warm, Cold, Mixed, and PCH

Learn about the four main types of Autoimmune Hemolytic Anemia (AIHA): warm, cold (CAD), mixed, and PCH. Understand how your subtype determines your treatment.

02

Diagnosis & Lab Reports: Decoding the Coombs Test

Learn how to read your AIHA lab report. Understand the Coombs test (DAT), signs of hemolysis like LDH and haptoglobin, and what your results mean for care.

03

Treatment Pathways for AIHA: Choosing the Right Road

Learn about treatment options for Autoimmune Hemolytic Anemia (AIHA). Understand the different pathways for Warm AIHA and Cold Agglutinin Disease (CAD).

04

Living with AIHA: Complications, Monitoring, and Survivorship

Learn what to expect when living with AIHA. Understand how to monitor your baseline labs, spot the signs of a hemolytic crisis, and manage blood clot risks.

Common questions in this guide

What is Autoimmune Hemolytic Anemia (AIHA)?
AIHA is a rare blood disorder where your immune system mistakenly produces antibodies that attack and destroy your own healthy red blood cells faster than your body can replace them.
What are the most common symptoms of AIHA?
Common symptoms include extreme fatigue, shortness of breath, jaundice (yellowing of the skin or eyes), and dark, tea-colored urine as the body filters out destroyed red blood cells.
What causes AIHA to develop?
AIHA can happen on its own (primary AIHA) or be triggered by an underlying condition like lupus, blood cancers such as CLL, certain infections like COVID-19, or specific medications.
How is AIHA diagnosed?
Doctors typically diagnose AIHA using a blood test called the Direct Antiglobulin Test (DAT), also known as the Coombs test. This test identifies specific immune antibodies attached to your red blood cells.
What kind of doctor should I see for AIHA?
Because AIHA is a rare condition that mimics more common problems, it should be managed by a hematologist. This specialist focuses specifically on blood disorders and has the expertise to guide your treatment.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What specific subtype of AIHA do I have (warm, cold, or mixed), and how does that change my treatment plan?
  2. 2.What was the result of my Direct Antiglobulin Test (DAT), and did it show IgG, C3d, or both?
  3. 3.Is my AIHA considered 'primary' or 'secondary'? Have we ruled out underlying conditions like Lupus, CLL, or certain infections?
  4. 4.What are the goals of my initial treatment, and how will we monitor if my red blood cell count is stabilizing?
  5. 5.How many patients with AIHA have you treated, or should I also see a hematologist who specializes in rare blood disorders?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (23)
  1. 1

    Warm Autoimmune Hemolytic Anemia.

    Brodsky RA

    The New England journal of medicine 2019; (381(7)):647-654 doi:10.1056/NEJMcp1900554.

    PMID: 31412178
  2. 2

    Haemolytic anaemia: a consequence of COVID-19.

    Jawed M, Hart E, Saeed M

    BMJ case reports 2020; (13(12)) doi:10.1136/bcr-2020-238118.

    PMID: 33303503
  3. 3

    Autoimmune haemolytic anaemias.

    Michel M, Crickx E, Fattizzo B, Barcellini W

    Nature reviews. Disease primers 2024; (10(1)):82 doi:10.1038/s41572-024-00566-2.

    PMID: 39487134
  4. 4

    Autoimmune Hemolytic Anemia.

    Liebman HA, Weitz IC

    The Medical clinics of North America 2017; (101(2)):351-359 doi:10.1016/j.mcna.2016.09.007.

    PMID: 28189175
  5. 5

    The Clinical Pictures of Autoimmune Hemolytic Anemia.

    Packman CH

    Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie 2015; (42(5)):317-24 doi:10.1159/000440656.

    PMID: 26696800
  6. 6

    Refractory autoimmune hemolytic anemia in a systemic lupus erythematosus patient: A clinical case report.

    Crawford LR, Neparidze N

    Clinical case reports 2022; (10(3)):e05583 doi:10.1002/ccr3.5583.

    PMID: 35340637
  7. 7

    A Complex Case of Anti-e Mimicking Autoantibody Development Leading to Warm Autoimmune Hemolytic Anemia: Case Report.

    Hale I, Kunes KL, Sabile J, et al.

    Acta haematologica 2026; 1-6 doi:10.1159/000550486.

    PMID: 41824628
  8. 8

    Autoimmune hemolytic anemia in COVID-19 patients: A systematic review of 105 cases on clinical characteristics and outcomes.

    Ayyad M, Abu Alya W, Barabrah AM, et al.

    Clinical immunology (Orlando, Fla.) 2025; (277()):110512 doi:10.1016/j.clim.2025.110512.

    PMID: 40348041
  9. 9

    Rapidly Progressing Autoimmune Hemolytic Anemia in a Pediatric Patient With COVID-19.

    Kononowicz JE, Farhan Ali M, Palko W, et al.

    Cureus 2023; (15(9)):e45633 doi:10.7759/cureus.45633.

    PMID: 37868512
  10. 10

    A Rare Case of Successfully Treated Coombs Negative Immune Haemolytic Anaemia in Pregnancy.

    Saha PK, Kaur J, Bagga R, et al.

    Journal of clinical and diagnostic research : JCDR 2017; (11(2)):QD06-QD07 doi:10.7860/JCDR/2017/22274.9409.

    PMID: 28384940
  11. 11

    Diagnosis and Management of Autoimmune Hemolytic Anemias.

    Barcellini W, Fattizzo B

    Journal of clinical medicine 2022; (11(20)) doi:10.3390/jcm11206029.

    PMID: 36294350
  12. 12

    Clinical Profile and Outcome of Childhood Autoimmune Hemolytic Anemia: A Single Center Study.

    Thatikonda KB, Kalra M, Danewa A, et al.

    Indian pediatrics 2021; (58(8)):737-740.

    PMID: 33634793
  13. 13

    A Rare Case of Cold Antibody-Mediated Autoimmune Haemolytic Anaemia in Early Systemic Lupus Erythematosus.

    Rajaram H, Ganegoda S, Andev R

    Cureus 2025; (17(6)):e86842 doi:10.7759/cureus.86842.

    PMID: 40718204
  14. 14

    Autoimmune Hemolytic Anemia in Children: Clinical Profile and Outcome.

    Kar S, Kumar CGD, Kar R, Basavarajegowda A

    Indian journal of pediatrics 2024; (91(2)):143-148 doi:10.1007/s12098-022-04469-6.

    PMID: 36787020
  15. 15

    Prevalence of systemic lupus erythematosus in autoimmune hemolytic anemia patients based on coombs test results.

    Harahap RIM, Ardiningrum T, Pamela Y, et al.

    European journal of medical research 2025; (30(1)):351 doi:10.1186/s40001-025-02601-8.

    PMID: 40312681
  16. 16

    Autoimmune haemolytic anaemia and antiphospholipid antibodies in paediatrics: a systematic review and meta-analysis.

    Merashli M, Arcaro A, Graf M, et al.

    Clinical rheumatology 2021; (40(5)):1967-1973 doi:10.1007/s10067-020-05436-2.

    PMID: 33006737
  17. 17

    Immune-mediated hemolytic anemia and thrombocytopenia in clonal B-cell disorders: a review.

    Durani U, Go RS, Kay NE

    Clinical advances in hematology & oncology : H&O 2018; (16(10)):670-676.

    PMID: 30543597
  18. 18

    Autoimmune Hemolytic Anemia in Chronic Lymphocytic Leukemia: A Comprehensive Review.

    Autore F, Pasquale R, Innocenti I, et al.

    Cancers 2021; (13(22)) doi:10.3390/cancers13225804.

    PMID: 34830959
  19. 19

    Warm Autoimmune Hemolytic Anemia Associated With Asymptomatic SARS-CoV-2 Infection.

    Liput JR, Jordan K, Patadia R, Kander E

    Cureus 2021; (13(3)):e14101 doi:10.7759/cureus.14101.

    PMID: 33927918
  20. 20

    Difficult Cases of Autoimmune Hemolytic Anemia: A Challenge for the Internal Medicine Specialist.

    Fattizzo B, Giannotta JA, Serpenti F, Barcellini W

    Journal of clinical medicine 2020; (9(12)) doi:10.3390/jcm9123858.

    PMID: 33261016
  21. 21

    Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting.

    Jäger U, Barcellini W, Broome CM, et al.

    Blood reviews 2020; (41()):100648 doi:10.1016/j.blre.2019.100648.

    PMID: 31839434
  22. 22

    Role of therapeutic plasma exchanges in refractory severe warm autoimmune hemolytic anemia: Presentation of two case reports.

    García-García I, Cid J, Palomino A, et al.

    Transfusion 2020; (60(11)):2753-2757 doi:10.1111/trf.16143.

    PMID: 33089904
  23. 23

    [The treatment strategies of autoimmune hemolytic anemia].

    Yue WR, Wu T, Wang XQ

    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 2024; (45(6)):615-620 doi:10.3760/cma.j.cn121090-20231027-00236.

    PMID: 39134500

This page provides an educational overview of Autoimmune Hemolytic Anemia (AIHA). It is not a substitute for professional medical advice, diagnosis, or treatment from a qualified hematologist.

Get notified when new evidence is published on Autoimmune hemolytic anemia.

We monitor PubMed for new peer-reviewed studies on this topic and email a short summary when something meaningful changes.