Introduction

Evans syndrome is a rare autoimmune disorder in which the body’s immune system mistakenly attacks its own blood cells. It is a combination of two conditions: autoimmune haemolytic anaemia (AIHA), the destruction of red blood cells and immune thrombocytopoenia (ITP), the destruction of platelets.

In some cases, white blood cells (neutrophils) may also be affected in this condition. Blood cells are needed for carrying oxygen, stopping bleeding, and fighting against infections. Evans syndrome causes serious health problems if not treated early. This makes first-line therapies extremely important. In this article, we are going to discuss the first-line treatments of Evans syndrome. ¹

Understanding Evans syndrome

The primary issue in Evans syndrome is the autoimmune function of the body. The immune system produces autoantibodies that mistakenly target and destroy healthy blood cells, which leads to the following conditions:

• Anaemia: the loss of red blood cells causes tiredness, shortness of breath, dizziness and pale skin. People with AIHA, especially those over 60 years of age, may have a higher risk of heart issues (like heart attacks) or stroke
• Thrombocytopoenia: due to low platelet levels, it leads to easy bruising, bleeding gums, or nosebleeds

There are some challenges in the management of Evan syndrome due to relapse and its chronic nature. Symptoms may improve with treatment but can return, making it difficult for long-term management. In this unpredictable situation, physicians often face challenges in balancing effective treatment with safety.¹

Goals of first-line therapy

The first goal in treatment is to immediately stabilise the condition of the individual. This includes the following:

• Stopping the destruction of red blood cells and platelets
• Reducing risks of severe bleeding and severe anaemia
• Helping the immune system regain its balance

Corticosteroids as first-line treatment

Corticosteroids (steroids) are usually the very first treatment physicians prescribe. 

Steroids calm the immune system, reducing inflammation and slowing down the production of harmful autoantibodies. Commonly used steroids are prednisone and dexamethasone. Most patients respond well within the first few weeks, but the duration of response can vary, and more than half relapse, making it essential to use additional or alternative treatment options. 

Long-term use of corticosteroids can cause problems such as bone thinning (osteoporosis), diabetes, infections, insomnia, weight gain, high blood pressure, oedema and in children, slowed growth. Because of this, physicians usually try to limit the duration of steroid use.^1,2

Intravenous immunoglobulin (IVIG) in Evans syndrome as therapy

Intravenous immunoglobulin (IVIG) can be used solely as first-line therapy when steroids do not respond well or are contraindicated. IVIG is made from healthy donors’ antibodies. It helps block the harmful immune response and protects blood cells from being destroyed. It is used in urgent cases and if the patient has a very low platelet count. IVIG is delivered through a vein (intravenously) in the hospital. It usually works faster than steroids, raising platelet counts within a few days. However, its effect is often temporary.

Some people experience side effects from IVIG, and they include:

• Headaches
• Fever
• Vomiting
• Fatigue
• Hepatitis
• Arthritis
• Rare reactions such as aseptic meningitis

Moreover, they might increase the risk of thrombosis that is already high during isolated AIHA, and might be similar in Evan syndrome. However, IVIG is an expensive treatment, which can limit its use.^3,4

Comparison of steroids and IVIG

Both of the treatments are effective on their own, but IVIG works faster, especially on platelets, while steroids provide longer-lasting results. Steroids may control the disease for weeks or months, but adverse effects limit long-term use. On the other hand, IVIG often wears off sooner. In severe cases, physicians may use both steroids and IVIG together to get better and sustained results in patients. Treatment does not stop at giving medicines; ongoing care and regular follow-up are equally important.

Regular blood tests like complete blood count (CBC), platelets, and neutrophil count, along with markers of red cell destruction, are important. As both steroids and IVIG influence the immune system, patients become more susceptible to infections; therefore, preventing any infection is essential.

Blood transfusions may be needed in severe anaemia, and antibiotics may be given to prevent infections. Doctors also recommend a nutritious diet, adequate sleep, and overall lifestyle guidance for managing this condition.

FAQs

How serious is Evans syndrome?

It can be very serious because blood cells are essential for carrying oxygen, preventing bleeding, and fighting infections. Without early treatment, patients may face complications like severe anaemia, dangerous bleeding or infections.

Does Evans syndrome come back after treatment?

Yes. Evans syndrome is often chronic and relapsing. Symptoms may improve after treatment, but can return, which makes long-term management and follow-up very important.

Can lifestyle changes help with Evans syndrome?

Yes. While medicines are the main treatment, physicians also recommend the following lifestyle management.

• Eating a healthy diet
• Getting enough rest
• Preventing infections through good hygiene
• Attending regular follow-up visits

Summary

Evans syndrome is a rare autoimmune disorder where the immune system attacks the body’s own blood cells. It involves two conditions: autoimmune haemolytic anaemia (AIHA), leading to the destruction of red blood cells, and immune thrombocytopenia (ITP), causing low platelet levels. The disease can also affect white blood cells and can lead to severe health issues if not treated promptly. The primary problem in Evans syndrome is the production of autoantibodies that destroy healthy blood cells. This results in symptoms such as tiredness, shortness of breath, dizziness, and pale skin due to anaemia, as well as easy bruising and bleeding due to low platelet levels. Managing Evans syndrome can be challenging because symptoms may improve with treatment but can return, making long-term treatment difficult. The first goal of treatment is to stabilise the patient’s condition by stopping the destruction of red blood cells and platelets, reducing severe bleeding risks, and helping the immune system. Corticosteroids, like prednisone and dexamethasone, are typically the first treatment choice, calming the immune system and reducing inflammation.

However, long-term use can lead to side effects like osteoporosis and weight gain, so their use is limited. Intravenous immunoglobulin (IVIG) can be an alternative treatment, especially when steroids are ineffective or not suitable. IVIG is made from antibodies from healthy donors and can work quickly to raise platelet counts, but has temporary effects. Both treatments have their advantages and disadvantages: IVIG works faster, while steroids provide more prolonged benefits but with potential side effects. Regular monitoring and follow-up care are crucial, and supportive measures like blood transfusions and dietary guidance may also be necessary.