Autoimmune Hemolytic Anemia: Causes, Types & Treatment

Autoimmune hemolytic anemia (AIHA) is a rare red blood cell disorder that affects the immune system. It is an autoimmune disorder in which the body produces antibodies (immunoglobulin G) that destroy red blood cells. Autoimmune hemolytic anemia can develop rapidly or over time, but it often goes away by itself.

AIHA causes the body’s immune system to destroy red blood cells faster than they can be replaced. Normally, the bone marrow produces new red blood cells every 3 weeks. But if you have AIHA, it is only possible to replace a small number of these new cells before more become damaged by the antibodies produced by your immune system. If replacement levels fall below a certain level, you may be anemic.

Causes of AIHA

White blood cells form part of your immune system, which has several roles. First, they fight off infections by producing special cell-surface proteins called antibodies. Antibodies attach to bacteria and viruses, destroying them so they cannot infect other parts of the body or spread to other people. Secondly, white blood cells (also called leukocytes) are involved in blood clotting. They gather at the site of injury and form a clot that prevents further bleeding.

Types of AIHA

There are two classifications of AIHA warm- cold, primary, and secondary.

Warm AIHA

The primary cause of the symptoms associated with warm hemolysis is IgG antibodies. These bind red blood cells at normal body temperature, about 98.6°F (37°C). The antibodies are usually weakly active and rarely cause symptoms. In some individuals, however, it can trigger a severe reaction that leads to a condition called hemolytic uremic syndrome (HUS).

Cold AIHA

Cold hemolysis is an autoimmune reaction, which means it can be triggered by a fight-or-flight response. It usually occurs when the blood is exposed to cold temperatures, specifically 32° to 39.2°F (0° to 4°C). Symptoms include tiredness and dizziness, pale or yellowing skin, pain in the chest and the backs of the legs, vomiting or diarrhea, pain, and blue coloring in your hands and feet. If you experience these symptoms after exposure to cold temperatures or a viral infection, you may have cold hemolysis.

Primary or Secondary AIHA

AIHA is a spectrum. At one end of the spectrum, there is primary AIHA, which occurs when there is no sign of any underlying condition. This can be any autoimmune disease or any condition that weakens the immune system. At the other end of the spectrum, there is secondary AIHA. Secondary AIHA occurs when there is a link with another condition such as systemic lupus erythematosus (SLE), rheumatoid arthritis, Hashimoto’s thyroiditis, or other disorders that weaken the immune system and may trigger changes that lead to AIHA. Common viruses that can trigger those changes include mumps, rubella, and Epstein-Barr virus. Viruses are not usually a primary cause because many people have both types of antibodies and anemia at the same time without having AIHA. Radiation therapy may cause this situation to develop occasionally if you have extra-pulmonary sarcoidosis causing pulmonary fibrosis which would prevent normal tissue regeneration leading to more serious diseases like SLE or systemic lupus erythematosus (SLE).

Symptoms

Common symptoms of AIHA are:

• A low-grade fever
• Weakness and tiredness
• Difficulty thinking and concentrating
• Paleness
• Rapid heartbeat
• Shortness of breath
• Yellowing skin, or jaundice
• Dark urine
• Muscle pains
• Headaches
• Nausea, vomiting, and diarrhea
• Lightheadedness when standing up
• Difficulty breathing
• A sore tongue
• Heart palpitations or a rapid heartbeat

Diagnosis

To determine whether you have anemia, your doctor will talk to you about your past medical history. They will also ask about medications and other medicines that you may take. They will also request a CBC test: a blood test to look for signs of anemia. A CBC is often ordered if you have any unexplained symptoms that could be related to anemia, such as Low energy levels, Low concentration, and lack of focus, especially at work or school Weight loss or unusual appetite Weakness Shortness of breath. The presence of red blood cells in your urine to rule out infection.

There are a few ways to check your red blood cell count such as:

• Reticulocyte count– This measures the number of young red blood cells in your body. A high reticulocyte count means your bone marrow is making a lot more cells to replace the ones your body has destroyed.

• Coombs test- The doctor will do this test to see if your body is making antibodies against red blood cells.

• Peripheral smear- Your doctor will check your red blood cells under a microscope for signs of blood cell destruction.

• Bilirubin test- Levels of this substance rise when blood cells are destroyed.

• Haptoglobin test- This protein clears debris from damaged red cells out of the bloodstream. Levels will be low when it is being used heavily.

• Cold agglutinin titer (CAT)- The doctor may test your level of these antibodies that attack red blood cells at cold temperatures by adding it to a patient’s sample and looking at how it reacts to certain substances such as proteins found in catheters and canals used during surgery or heart bypass surgery procedures, etc.

Treatment

Anemia of iron deficiency is a common condition that can affect anyone. It is not serious, but it can cause fatigue and make it harder to concentrate.

If you think your anemia may be due to iron deficiency, talk to a doctor or nurse about your symptoms and medical history. They will ask about your overall health and physical activity, as well as medications or supplements you take regularly. They will also review your symptoms and possible causes of anemia—such as blood loss from menstruation or surgery. If there is no underlying cause for your anemia, they may recommend treatment options without testing you first.

The treatment options depend on many factors including:

• The person’s age, overall health, and medical history.
• How severe the anemia is
• The cause of the condition
• The individual’s tolerance for specific treatments

Medication

The immune system plays a vital role in our ability to stay healthy. When it attacks the body, it can cause serious complications, including anemia and low red blood cell counts. If you have AIHA, your doctor may prescribe corticosteroids or cortisone-like drugs to weaken the immune response.

This is the first type of treatment for people with primary AIHA, and it can help to improve symptoms in many common types of AIHA. In severe cases, and if these drugs do not work, a doctor may prescribe other drugs that suppress the immune response, known as immunosuppressive therapy.

This drug treatment helps to lower the body’s immune response. The drugs help to prevent the immune system from attacking its bone marrow. Doing so allows the marrow stem cells to grow, and this can increase red blood counts. However, both cortisone and immunosuppressant drugs can have adverse effects.

AIHA in children

AIHA is a condition that causes fatigue and weakness. The fatigue makes it hard for children to get enough sleep and make it through the day. This weakness makes it hard for them to participate in their usual daily activities, such as playing with friends or going to school.

Children who have AIHA may also have cold symptoms like a runny nose, sneezing, and sore throat.

According to the University of Chicago, fewer than 0.2 people in every 100,000 have AIHA before the age of 20 years. The highest rates are in preschool-age children.

When AIHA occurs in children, it is usually due to a virus or infection—but it can also be triggered by certain medications or illnesses such as asthma or allergies. Some cases of AIHA do not respond as well as others when treated with antibiotics; therefore, doctors often recommend starting treatment with antiviral medications before antibiotics are prescribed.

A doctor will discuss a specific treatment plan that includes taking the medication regularly at home until symptoms improve or go away completely.

Prevention

AIHA is a condition that affects the body’s ability to produce red blood cells. The most common causes of AIHA are cancer and viral infections, but they can also be caused by medications or other medical conditions.

You can prevent some types of AIHA by monitoring people who have a viral infection or who use certain medications, to ensure that AIHA does not develop. However, severe anemia can worsen many problems, such as heart and lung disease. People should contact a doctor if they experience any symptoms that may indicate AIHA.

AIHA is a condition that occurs when the adrenal gland does not produce enough cortisol. Cortisol is a hormone that activates the immune system and increases blood pressure, helps regulate blood sugar levels, and keeps your body from getting fat.

The outlook for AIHA is good. The condition usually lasts for a limited time and rarely leads to long-term complications. If it occurs during the teenage years, it can become a long-term condition. However, medical treatment can help reduce the impact.