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Autoimmune hemolytic anemia (AIHA) is an autoimmune disease where the immune system mistakenly attacks your red blood cells (RBCs), causing anemia. Symptoms of AIHA can range from mild to life-threatening. AIHA is associated with increased mortality (likelihood of death), but it can be difficult to understand what that means for an individual.
Different factors can influence your chance of recovery and the likely course of the disease — known as your prognosis. Read on to learn more about the factors that can affect your outlook with AIHA.
AIHA is a rare disease — about 1.77 people out of 100,000 develop AIHA every year, according to a study in Immunity & Ageing. People may be more likely to develop AIHA if they:
When people are diagnosed with AIHA, their doctor may give them a prognosis, predicting how likely their condition will respond to treatment and recur (come back after treatment), as well as the likelihood of death.
Severe anemia is one of the most important factors doctors will consider to predict your prognosis. Hemoglobin (Hb) is a protein in red blood cells needed to deliver oxygen to tissues of the body. A level of Hb lower than 8 grams per deciliter (g/dL) is associated with more severe AIHA cases and a higher chance of recurrence. Hb lower than 6 g/dL is associated with a five- to eight-fold increased risk of death.
Other factors associated with an increased risk of death include:
There are two main types of AIHA — warm autoimmune hemolytic anemia and cold AIHA. Warm AIHA is the most common type, making up about 80 percent of cases in adults, according to Cleveland Clinic.
AIHA is also classified based on what causes it. Primary AIHA refers to AIHA that develops without an obvious underlying condition to cause it — this may also be called idiopathic. Secondary AIHA is associated with another condition or a medication. Many studies focus on the outcomes of primary and secondary AIHA instead of warm and cold AIHA.
A 37-year-long study on AIHA survival in a Danish population found that more than 80 percent of people survived at least one year after being diagnosed with primary AIHA and almost 70 percent survived at least one year after being diagnosed with secondary AIHA.
This study also measured median survival for primary and secondary AIHA. Median survival refers to the length of time from diagnosis when half of the people in the study group are still alive. Median survival for primary AIHA was 9.8 years; median survival for secondary AIHA was significantly shorter at 3.3 years.
The prognosis of AIHA is generally good for people diagnosed before age 30. In the Danish study, people who were diagnosed with primary AIHA had about the same life expectancy as the general population. However, the life expectancy of people diagnosed with AIHA when they were over the age of 30 was significantly lower.
Studies have shown that people are more likely to develop AIHA when they’re older. The incidence of AIHA is about 10 times higher in people over 75 compared to people under 50, according to a 2021 study in the American Journal of Hematology (AJH).
AIHA may be more dangerous to older people because they’re more likely to develop other comorbidities (co-occurring medical conditions) that can complicate AIHA or its treatment. For example, people with diabetes often have a worse prognosis for AIHA than people without diabetes.
People with AIHA have an increased risk of death from several different causes. Studies show that the first year after an AIHA diagnosis, especially the first 100 days, is associated with the highest risk of death from any cause.
The most common causes of death in people with AIHA include thrombosis (blood clots inside blood vessels), cardiovascular problems, cancer, and infection.
A 2022 study found that 11 percent of people with AIHA also had thrombosis, potentially life-threatening depending on where blood clots develop. The risk of thrombosis is highest in the first year after diagnosis. The AJH study found that 6 percent of people with primary AIHA were hospitalized for thrombosis in the first year after diagnosis. The most common type of thrombosis was a pulmonary embolism — a blocked blood vessel in the lung.
Studies have found that the following factors increase the risk of thrombosis:
People with AIHA may be more likely to experience cardiovascular (heart) problems such as a heart attack or coronary artery disease. The Danish study found that cardiovascular-related death was the most frequent cause of death in people with primary AIHA in the first year and in the first five years after diagnosis.
Hematological (blood) cancers are associated with secondary AIHA. The Danish study found that about 8 percent of people with secondary AIHA died from blood cancers such as CLL within the first 100 days after diagnosis. One year after diagnosis of primary AIHA, about 1 percent of people had died from blood cancer.
The Danish study found that 5 percent of people with AIHA died from an infection within around four years of diagnosis. Infections can be especially dangerous for people with a history of AIHA because it can trigger a relapse of AIHA symptoms. The AJH study found that almost 23 percent of people with primary AIHA were hospitalized for an infection in the first year after diagnosis.
Treatments for AIHA can increase your risk of infections because they can hamper aspects of your immune system, causing immunodeficiency. These include medications such as rituximab, cyclosporine, and steroids such as prednisone. Additionally, having a splenectomy — surgical removal of the spleen — may increase your risk of infection.
People with diabetes may be at an even higher risk of death from infection. A 2009 study in Hematology found that infection was the most common cause of death among 53 people with diabetes and AIHA.
In the past 40 years, improvements in treatment options, supportive care, and the management of comorbidities have led to an increased life expectancy for people with AIHA. The introduction of rituximab to treat AIHA in 1997 appears to have had a large impact on median survival.
Recent research continues to provide new insights. Researchers for the Danish study found that median survival increased from 8.18 years in the 1980s to 12.6 years in 2000. Secondary AIHA median survival increased from 1.12 years in the 1980s to 3.89 years in 2000. The median survival has stayed about the same since the year 2000.
The prognosis of AIHA has improved over the years. Talk to your doctor about your treatment options, and make sure to stick with your therapy.
While you can’t prevent AIHA, making changes to your diet may help reduce your risk of complications such as heart disease. Inflammation is also associated with thrombosis, so an anti-inflammatory diet rich in fruits, vegetables, and good fats may help prevent dangerous blood clots.
Other lifestyle changes can help you decrease your risk of heart disease and diabetes, such as:
You can reduce your risk of infection by:
On myAIHAteam — the social network for people with AIHA and their loved ones — members come together to ask questions, give advice, and share their stories with others who understand life with AIHA.
Are you living with AIHA? Have you discussed prognosis with your doctor? Share your experience in the comments below, or start a conversation by posting on your Activities page.
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Richard LoCicero, M.D.
has a private practice specializing in hematology and medical oncology at the Longstreet Clinic Cancer Center, in Gainesville, Georgia. Review provided by VeriMed Healthcare Network.
Learn more about him here.
Amanda Jacot, PharmD
earned a Bachelor of Science in biology from the University of Texas at Austin in 2009 and a Doctor of Pharmacy from the University of Texas College of Pharmacy in 2014.
Learn more about her here.
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