About Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia treatment, Types of autoimmune hemolytic anemia, Autoimmune hemolytic anemia symptoms

Overview autoimmune hemolytic anemia

Autoimmune hemolytic anemia is a rare red blood cell disorder and d an immune disorder. It happens when the body produces antibodies that destroy the red blood cells.

Autoimmune hemolytic anemia, or AIHA, is a rare type of anemia. When you have anemia, your bone marrow doesn’t make enough red blood cells. Or these cells don’t work as well as they should.

Red blood cells carry oxygen to your body. When you have too few red blood cells, your body can’t get enough oxygen, leaving you feeling tired or short of breath.

Hemolytic anemia develops when there are not enough red blood cells because the body destroys them sooner than it should. Red blood cells carry oxygen around the body.

Autoimmune hemolytic anemia (AIHA), or immune hemolytic anemia, happens when the immune system does not work properly. It mistakes red blood cells for unwanted substances and attacks them, causing them to die early. This leaves a person without enough red blood cells.

Normally, red blood cells live in the body for 100 to 120 days. However, in severe cases of AIHA, the cells may remain only for a few days.

In children, it is a rare condition that is usually temporary. In some adults, however, AIHA can be a long-term condition that returns frequently.

History

“Blood-induced icterus” produced by the release of massive amounts of a coloring material from blood cells followed by the formation of bile was recognized and described by Vanlair and Voltaire Masius’ in 1871. About 20 years later, Hayem distinguished between congenital hemolytic anemia and an acquired type of infectious icterus associated with chronic splenomegaly.

In 1904, Donath and Landsteiner suggested a serum factor was responsible for hemolysis in paroxysmal cold hemoglobinuria. French investigators led by Chauffard stressed the importance of red-cell autoagglutination in patients with acquired hemolytic anemia. In 1930, Lederer and Brill described cases of acute hemolysis with rapid onset of anemia and rapid recovery after transfusion therapy.

These hemolytic episodes were thought to be due to infectious agents. A clear distinction between congenital and acquired hemolytic anemia was not drawn, however, until Dameshek and Schwartz in 1938, and, in 1940, they demonstrated the presence of abnormal hemolysins in the sera of patients with acquired hemolytic anemia and postulated an immune mechanism.

During the past three decades, studies defining red-cell blood groups and serum antibodies have produced diagnostic methods that have laid the basis for immunologic concepts relevant to many of the acquired hemolytic states. Of these developments, the antiglobulin test described by Coombs, Mourant, and Race in 1945 has proved to be one of the more important, useful tools now available for the detection of immune hemolytic states.

This technique demonstrated that a rabbit antibody against human globulin would induce agglutination of human red cells “coated with an incomplete variety of rhesus antibodies”. C. Moreschlit had used the same method in 1908 in a goat antirabbit-red-cell system. The test was premature and was forgotten. In 1946, Boorman, Dodd, and Loutit applied the direct antiglobulin test to a variety of hemolytic anemias and laid the foundation for the clear distinction of autoimmune from congenital hemolytic anemia.

A hemolytic state exists whenever the red cell survival time is shortened from the normal average of 120 days. Hemolytic anemia is the hemolytic state in which anemia is present, and bone marrow function is inferentially unable to compensate for the shortened life-span of the red cell. Immune hemolytic states are those, both anemic and nonanemic, which involve immune mechanisms consisting of antigen-antibody reactions.

These reactions may result from unrelated antigen-antibody complexes that fix to an innocent-bystander erythrocyte, or from related antigen-antibody combinations in which the host red cell or some part of its structure is or has become antigenic. The latter type of antigen-antibody reaction may be termed “autoimmune”, and hemolytic anemias so produced are autoimmune hemolytic anemias.

In children

In general, AIHA in children has a good prognosis and is self-limiting. However, if it presents within the first two years of life or in the teenage years, the disease often follows a more chronic course, requiring long-term immunosuppression, with serious developmental consequences. The aim of therapy may sometimes be to lower the use of steroids in the control of the disease. In this case, splenectomy may be considered, as well as other immunosuppressive drugs. Infection is a serious concern in patients on long-term immunosuppressant therapy, especially in very young children (less than two years).

What is AIHA?

Red blood cells are made in the spongy material called bone marrow deep inside your bones. These blood cells normally live for about 120 days.

If you have autoimmune hemolytic anemia, your body’s immune system attacks and destroys red blood cells faster than your bone marrow can make new ones. Sometimes these red blood cells live for only a few days.

Most people who get AIHA are middle-aged or older. It’s rare in children, and it usually shows up soon after a viral illness and goes away on its own. If your teenager gets it, it could be a sign they have some other health problem.

Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia is a group of disorders characterized by a malfunction of the immune system that produces autoantibodies, which attack red blood cells as if they were substances foreign to the body.

  • Some people have no symptoms, and other people are tired, short of breath, and pale.
  • Severe disease may cause jaundice or abdominal discomfort and fullness due to splenomegaly (an enlarged spleen).
  • Blood tests are used to detect anemia and determine the cause of the autoimmune reaction.
  • Treatment is corticosteroids or other drugs that suppress the immune system and sometimes, splenectomy (surgical removal of the spleen).

Autoimmune hemolytic anemia is an uncommon group of disorders that can occur at any age. These disorders affect women more often than men. About half of the time, the cause of autoimmune hemolytic anemia cannot be determined (idiopathic autoimmune hemolytic anemia). Autoimmune hemolytic anemia can also be caused by or occur with another disorder, such as systemic lupus erythematosus (lupus) or lymphoma, and it can be due to the use of certain drugs, such as penicillin.

Destruction of red blood cells by autoantibodies may occur suddenly, or it may develop gradually. If caused by a virus, the destruction may stop after a period of time. In other people, red blood cell destruction persists and becomes chronic. There are two main types of autoimmune hemolytic anemia:

Warm antibody hemolytic anemia: The autoantibodies attach to and destroy red blood cells at normal body temperature.

Cold antibody hemolytic anemia (cold agglutinin disease): The autoantibodies become most active and attack red blood cells only at temperatures well below normal body temperature.

Paroxysmal cold hemoglobinuria (Donath-Landsteiner syndrome) is a rare type of cold antibody hemolytic anemia. Destruction of red blood cells results from exposure to cold. Red blood cells may be destroyed even when cold exposure is limited to a small area of the body, such as when the person drinks cold water or washes hands in cold water.

An antibody binds to red blood cells at low temperatures and causes the destruction of red blood cells within arteries and veins after warming. It occurs most often after a viral illness or in otherwise healthy people, although it occurs in some people with syphilis. The severity and rapidity of the development of the anemia vary.

Abstract

The diagnosis of autoimmune hemolytic anemia (AIHA) can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis and then determine the immune nature of hemolysis with the direct anti-globulin test. Once alternative causes for these findings have been excluded, AIHA is established, and the clinician must search for secondary causes, as well as identify the type of AIHA.

Rituximab is now the preferred second-line treatment for primary warm AIHA and first-line treatment for primary cold agglutinin disease (CAD), either as monotherapy or combined with bendamustine. Complement inhibitors have shown utility in stabilizing AIHA patients with acute severe hemolysis. Future prospects are discussed and include the C1s inhibitor BIVV009 (sutimlimab) that is now entering phase 3 studies for CAD.

Summary

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. 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. 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. Treatment may include corticosteroids such as prednisone, splenectomy, immunosuppressive drugs, and/or blood transfusions.

Autoimmune hemolytic anemia (AIHA) occurs when antibodies directed against the person’s own red blood cells (RBCs) cause them to burst (lyse), leading to an insufficient number of oxygen-carrying red blood cells in the circulation. The lifetime of the RBCs is reduced from the normal 100–120 days to just a few days in serious cases.

The intracellular components of the RBCs are released into the circulating blood and into tissues, leading to some of the characteristic symptoms of this condition. The antibodies are usually directed against high-incidence antigens, therefore they also commonly act on allogenic RBCs (RBCs originating from outside the person themselves, e.g. in the case of a blood transfusion). AIHA is a relatively rare condition, affecting one to three people per 100,000 per year. Autoimmune hemolysis might be a precursor of later-onset systemic lupus erythematosus.

The terminology used in this disease is somewhat ambiguous. Although MeSH uses the term “autoimmune hemolytic anemia”, some sources prefer the term “immune hemolytic anemia” so drug reactions can be included in this category.

The National Cancer Institute considers “immune hemolytic anemia”, “autoimmune hemolytic anemia”, and “immune-complex hemolytic anemia” to all be synonyms.

  • Warm-antibody type
  • Primary
  • Secondary (lymphoproliferative disorders, autoimmune disorders)
  • Cold-antibody type (anemia)
  • Primary cold agglutinin disease
  • Secondary cold agglutinin syndrome
  • Associated with malignant disease
  • Acute, transient, infection-associated (acute cold antibody-mediated AIHA complicating Mycoplasma pneumonia or viral infections)
  • Chronic (lymphoproliferative disorders)
  • Paroxysmal cold hemoglobinuria)
  • Idiopathic
  • Secondary
  • Acute, transient (Infections other than syphilis)
  • Chronic (syphilis)
  • Mixed cold- and warm-antibody type
  • Idiopathic
  • Secondary (lymphoproliferative disorders, autoimmune disorders)
  • Drug-induced immune hemolytic anemia
  • Autoimmune type
  • Drug absorption type

Autoimmune hemolytic anemia (AIHA) is an uncommon entity that presents diagnostic, prognostic, and therapeutic dilemmas despite being a well-recognized entity for over 150 years. This is because of significant differences in the rates of hemolysis and associated diseases and because there is considerable clinical heterogeneity. In addition, there is a lack of clinical trials required to refine and update standardized and evidence-based therapeutic approaches.

To aid the clinician in AIHA management, we present four vignettes that represent and highlight distinct clinical presentations with separate diagnostic and therapeutic pathways that we use in our clinical practice setting. We also review the parameters present in diagnostic testing that allow for prognostic insight and present algorithms for both diagnosis and treatment of the AIHA patient in diverse situations.

This is done in the hope that this review may offer guidance in regard to personalized therapy recommendations. A section is included for the diagnosis of suspected AIHA with negative test results, a relatively infrequent but challenging situation, in order to assist in the overall evaluation spectrum for these patients.

Autoimmune hemolytic anemia (AIHA) is defined as the increased destruction of red blood cells (RBCs) in the presence of anti-RBC autoantibodies and/or complement. Classification of AIHA is based on the optimal auto-RBC antibody reactivity temperatures and includes warm, cold-reactive, mixed AIHA, and drug-induced AIHA subtypes.

AIHA is a rare disease, and recommendations for transfusion are based mainly on results from retrospective data and relatively small cohort studies, including heterogeneous patient samples or single case reports. In this article, we will review the challenges and solutions to safely transfuse AIHA patients. We will reflect on the indication for transfusion in AIHA and the difficulty in the accomplishment of immunohistological procedures for the selection of the safest and most compatible RBC units.

Introduction

Autoimmune hemolytic anemia (AIHA) is defined as the increased destruction of red blood cells (RBCs) in the presence of anti-RBC autoantibodies and/or complement. The annual incidence of AIHA range from 1 to 3 in 100,000 to 1 in 25,000 individuals. The variability likely reflects the use of different criteria for diagnosis of AIHA that often confounds comparison among studies. AIHA affects people of all ages, but it is more common in adults than in children. Symptoms and physical findings reflect the premature destruction of RBCs with inadequate compensation of bone marrow and the secondary effect of hemolysis. The diagnosis of AIHA is usually simple and based on the presence of hemolytic anemia and serological evidence of anti-RBC autoantibodies detected by the direct antiglobulin test (DAT).

Since AIHA is a rare disease, treatment recommendations for patients with this condition, including transfusional support are based mainly on results from retrospective data and relatively small cohort studies, including heterogeneous patient samples or single case reports. Moreover, a frequent finding in immunohematology is the presence of anti-RBC autoantibodies without clinical symptoms of hemolysis.

Immune hemolytic anemias have been classified based on different criteria; however, AIHA is often classified by the optimal temperature at which the autoantibodies bind to the patient’s RBCs in vivo. In this context, the classification system includes warm AIHA (WAIHA), cold AIHA (CAIHA) (which includes cold agglutinin syndrome [CAS] and paroxysmal cold hemoglobinuria [PCH]), mixed AIHA (MAIHA), and drug-induced immune hemolytic anemia (DIIHA) In WAIHA, the autoantibodies present in the serum react optimally with human RBCs at 37ºC, while CAIHA is mediated by cold autoantibodies exhibiting affinity for RBCs at temperatures below 37ºC, and MAIHA patients exhibit both cold- and warm-reactive autoantibodies.

Types of autoimmune hemolytic anemia

Autoimmune hemolytic anemia is classified in a few different ways:

  • Primary AIHA: Appears by itself
  • Secondary AIHA: Affects you because you have another illness. 

The disease is also classified by the temperature at which red blood cells are damaged:

  • Warm antibody hemolytic anemia: The immune reaction takes place at or above normal body temperature. 
  • Cold antibody hemolytic anemia: Red blood cells are destroyed when you’re exposed to cold.

Causes

The causes of AIHA are poorly understood. The disease may be primary, or secondary to another underlying illness. The primary illness is idiopathic (the two terms used synonymously). Idiopathic AIHA accounts for approximately 50% of cases.

Secondary AIHA can result from many other illnesses. Warm and cold type AIHA each have their own more common secondary causes. The most common causes of secondary warm-type AIHA include lymph proliferative disorders (e.g., chronic lymphocytic leukemialymphoma) and other autoimmune disorders (e.g., systemic lupus erythematosusrheumatoid arthritissclerodermaCrohn’s diseaseulcerative colitis).

Less common causes of warm-type AIHA include neoplasms other than lymphoid, and infection. Secondary cold type AIHA is also caused primarily by lymph proliferative disorders, but is also commonly caused by infection, especially by mycoplasma, viral pneumonia, infectious mononucleosis, and other respiratory infections. Less commonly, it can be caused by concomitant autoimmune disorders.

Drug-induced AIHA, though rare, can be caused by a number of drugs, including α-methyldopa and penicillin.

This is a type II immune response in which the drug binds to macromolecules on the surface of the RBCs and acts as an antigen. Antibodies are produced against the RBCs, which leads to complement activation. Complement fragments, such as C3a, C4a and C5a, activate granular leukocytes (e.g., neutrophils), while other components of the system (C6, C7, C8, C9) either can form the membrane attack complex (MAC) or can bind the antibody, aiding phagocytosis by macrophages (C3b). This is one type of “penicillin allergy”.

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.

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. Secondary causes of autoimmune hemolytic anemia include:

  • Autoimmune diseases, such as lupus
  • Chronic lymphocytic leukemia
  • Non-Hodgkin’s lymphoma and other blood cancers
  • Epstein-Barr virus
  • Cytomegalovirus
  • Mycoplasma pneumonia
  • Hepatitis
  • HIV

You can get autoimmune hemolytic anemia if you have an autoimmune disease like lupus. Normally when your immune system spots foreign invaders like bacteria and viruses, it makes proteins called antibodies to attack them.

Blood cells are made in the bone marrow.

There are three basic types:

  • White blood cells help the body to fight off fight infections.
  • Platelets help the blood to clot and prevent bleeding.
  • Red blood cells transport oxygen throughout the body in the form of hemoglobin.

White blood cells produce Trusted Source antibodies. Antibodies attach to red blood cells and travel throughout the body, fighting germs and other foreign substances that should not be there.

In AIHA, the body makes antibodies that attack the red blood cells because they think they are foreign or unwanted substances. They destroy the red blood cells, and this results in anemia.

This may happen because of:

  • exposure to certain toxins or chemicals, for example in medications
  • a complication of an infection
  • receiving a blood transfusion when the blood does not match the individual’s blood type
  • an unborn baby’s blood type being different from that of their mother
  • some types of cancer

When you have AIHA, your immune system makes antibodies that mistakenly attack your own red blood cells.

Other diseases and medications can also cause autoimmune hemolytic anemia. Some of these are:

  • Cancers, including chronic lymphocytic leukemia and non-Hodgkin’s lymphoma
  • Infections like Mycoplasma pneumonia
  • Medicines such as penicillin, methyldopa (Aldomet), quinine (Qualaquin), and sulfonamides
  • Viruses such as Epstein-Barr virus, cytomegalovirus, HIV, and hepatitis

Types and risk factors

There are two classifications for AIHA: warm versus cold and primary versus secondary.

The warm or cold classification depends on the type of antibodies involved.

Warm AIHA

Also called warm hemolysis, this involves IgG antibodies. These bind red blood cells at 98.6°F (37°C), or normal body temperature. This accounts for 80–90 percent of cases.

Symptoms usually appear gradually, over a period of several weeks to months. Sometimes, however, they can emerge suddenly, within a few days.

They include:

  • pale or yellowing skin
  • tiredness
  • dizziness
  • heart palpitations

Cold AIHA

This is also called cold hemolysis. In this type, IgM autoantibodies, or cold agglutinins, bind red blood cells when the blood is exposed to cold temperatures, specifically 32° to 39.2°F (0° to 4°C). This accounts for 10–20 percent of cases.

Cold temperatures or a viral infection can trigger symptoms, which include:

  • tiredness and dizziness
  • pale or yellowing skin
  • cold hands and feet
  • pain in the chest and the backs of the legs
  • vomiting or diarrhea
  • pain and blue coloring in the hands and feet
  • Raynaud’s disease
  • heart problems, such as arrhythmia, a heart murmur, an enlarged heart, or heart failure

Primary or secondary AIHA

AIHA can also be primary or secondary.

  • Primary AIHA is when there is no sign of any underlying condition.
  • Secondary AIHA is when there is a link with another condition.

Autoimmune diseases that have or may have a link with secondary AIHA include:

  • rheumatoid arthritis
  • systemic erythematous lupus (SLE)
  • Sjogren’s syndrome
  • ulcerative colitis
  • thyroid disease
  • Hashimoto’s thyroiditis
  • long-term kidney disease
  • other conditions that weaken the immune system

Some common viruses may trigger AIHA. Often, the antibodies and anemia go away once the infection has gone.

Viruses that appear to have a link with AIHA include:

  • mycoplasma pneumonia
  • Epstein-Barr virus (EBV)
  • measles
  • mumps
  • rubella
  • varicella, which causes chickenpox
  • hepatitis
  • HIV
  • cytomegalovirus

These viruses can trigger changes to antibodies that can lead to AIHA.

Types of drugs that, in rare cases, may trigger changes that lead to AIHA include:

  • penicillins
  • cephalosporins
  • tetracycline
  • erythromycin
  • acetaminophen
  • ibuprofen

A person can also inherit antibodies from their mother at birth, but this is rare.

Autoimmune hemolytic anemia symptoms

Symptoms of idiopathic AIHA

You may feel weak and short of breath if you develop sudden-onset idiopathic AIHA. In other instances, the condition is chronic and develops over time, so symptoms are less obvious.

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.

You may not have symptoms if the anemia is mild. If the problem develops slowly, symptoms that may occur first include:

  • Feeling weak or tired more often than usual, or with exercise
  • Headaches
  • Problems concentrating or thinking

If the anemia gets worse, symptoms may include:

  • Lightheadedness when you stand up
  • Pale skin color (pallor)
  • Shortness of breath
  • Sore tongue

The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Common symptoms of AIHA include:

  • 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

In both cases, symptoms may include one or more of the following:

  • increasing weakness
  • shortness of breath
  • rapid heartbeat
  • pale or yellow-colored skin
  • muscle pain
  • nausea
  • vomiting
  • dark-colored urine
  • a headache
  • abdominal discomfort
  • bloating
  • diarrhea

Diagnosis

A doctor will ask about symptoms and carry out a physical examination. They may then order some blood and urine tests to help with the diagnosis. They may refer you to a hematologist, a doctor who specializes in blood diseases. They’ll most likely discuss your past medical history, medications you take, and talk about your symptoms.

They’ll also request a blood test called a complete blood count, or CBC, to look for signs of anemia. This test measures:

  • The number of red blood cells, white blood cells, and platelets
  • The size of your red blood cells
  • The protein in your red blood cells that carries oxygen (hemoglobin)
  • How much space red blood cells take up in your blood (hematocrit)

Once blood tests show a person has anemia, doctors look for the cause. Doctors suspect increased destruction of red blood cells when a blood test shows an increase in the number of red blood cells that are immature (reticulocytes) or there is evidence of blood destruction on a blood smear (a test in which a drop of blood is spread on a slide and examined under a microscope).

Alternatively, a blood test may show an increased amount of a substance called bilirubin produced by the destruction of red blood cells and a decreased amount of a protein called haptoglobin, which binds the hemoglobin released from the destroyed red cells.

A low red blood cell count and low hemoglobin and hematocrit levels are signs of anemia.

Complete blood count

The complete blood count (CBC) measures the different parts that make up the blood.

It includes measuring the hemoglobin and hematocrit levels.

  • Hemoglobin is a protein that carries oxygen throughout the body.
  • Hematocrit shows how much space red blood cells take up.

Low levels of both can be a sign of anemia.

Coombs tests

These blood tests look for antibodies that may affect the red blood cells.

Reticulocyte test

This blood test measures the levels of reticulocytes, which are slightly immature red blood cells. It can determine whether the bone marrow is creating red blood cells at a suitable rate.

The range will be higher if the body has low hemoglobin levels due to bleeding or red cell destruction. High red blood cells production can be a sign of anemia.

Bilirubin test

The liver produces bilirubin, a yellow-colored substance that is present in bile. A blood test can measure the amount of bilirubin in the blood.

When blood cells die, hemoglobin enters the bloodstream. Hemoglobin, in turn, breaks down into bilirubin. This leads to jaundice when the eyes and skin take on a yellowish color.

High bilirubin levels in the blood can be a sign of anemia, liver damage, or another disease.

Haptoglobin test

Haptoglobin is a protein that the liver produces. Within the body, it connects a specific type of hemoglobin within the blood.

The amount of haptoglobin in the blood shows how fast red blood cells are being destroyed.

Cold agglutinins test

Cold agglutinin disease is a rare type of AIHA in which symptoms become worse when a person is in temperatures between 32º and 50º Fahrenheit.

Agglutinins are antibodies that cause red blood cells to clump together. Cold agglutinins are active at cold temperatures, and warm agglutinins are active at normal body temperatures.

Determining whether there are warm or cold agglutinins can sometimes help explain why the disorder is occurring.

Warm agglutinins can occur with:

  • certain infections, such as mycoplasma pneumonia
  • some medications, including penicillin

Autoimmune hemolytic anemia as the cause is confirmed when blood tests detect increased amounts of certain antibodies, either attached to red blood cells (direct antiglobulin or direct Coombs test) or in the liquid portion of the blood (indirect antiglobulin or indirect Coombs test). Other tests sometimes help determine the cause of the autoimmune reaction that is destroying red blood cells.

Autoimmune hemolytic anemia treatment

Plentiful literature exists regarding the treatment of AIHA. Efficacy of treatment depends on the correct diagnosis of either warm- or cold-type AIHA

When acquired autoimmune hemolytic anemia is secondary to other diseases, diagnosis and treatment of the underlying disorder usually bring marked improvement of the anemia. Mild cases may require no treatment. Individuals with more severe cases of warm antibody hemolytic anemia may be treated with oral steroids or intravenous hydrocortisone followed by divided daily oral doses of prednisone. Improvement usually occurs within five to ten days after treatment.

Warm-type AIHA is usually a more insidious disease, not treatable by simply removing the underlying cause. Corticosteroids are first-line therapy. For those who fail to respond or have recurrent disease, splenectomy may be considered. Other options for the recurrent or relapsed disease include immunosuppressants such as rituximab, danazol, cyclophosphamide, azathioprine, or cyclosporine.

Cold agglutinin disease is treated with avoidance of cold exposure. Patients with more severe disease (symptomatic anemia, transfusion dependence) may be treated with rituximab. Steroids and splenectomy are less efficacious in cold agglutinin disease.

Paroxysmal cold hemoglobinuria is treated by removing the underlying cause, such as infection.

Treatment options for AIHA depend on a number of factors. If the anemia is mild, it often passes without treatment. Between 70 and 80 percent of people need no treatment or minimal intervention.

If you have a disease like lupus that’s causing your anemia, your doctor will treat it first. If a medication is a cause, you’ll likely have to stop taking that medicine. If your AIHA is mild, you may not need treatment.

Doctors usually first prescribe steroids, such as hydrocortisone or prednisone, to stop your immune system from attacking your red blood cells. A medicine called rituximab may make steroids work even better.

If you don’t improve, you may need surgery to remove your spleen. That’s where much of the destruction of red blood cells takes place. 

Other medicines such as azathioprine (Imuran) and cyclophosphamide (Cytoxan) can be used to suppress the immune system. You may need a blood transfusion.

However, some people will need medication, surgery, or a blood transfusion.

Factors affecting the need for treatment include:

  • 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
  • how health providers expect the symptoms to progress

If there is an underlying cause—such as cancer, an infection, or the use of some medications—treating the condition or changing the medication may reduce the symptoms of AIHA.

Treatment options for IAIHA

People suspected of having sudden onset idiopathic AIHA will generally be hospitalized immediately because of its acute nature. Chronic cases may often come and go without explanation. It’s possible for the condition to improve without treatment.

Your doctor will monitor your blood glucose levels closely if you have diabetes. Diabetes is a major risk factor for deaths from infection as a result of treatment.

Steroids

The first-line treatment is typically steroids such as prednisone. They may help improve red blood cell counts. Your doctor will carefully monitor you to check that the steroids are working. Once your condition goes into remission, your doctor will try to wean you off of the steroids slowly. People with AIHA undergoing steroid therapy may need supplements during treatment. These could include:

  • bisphosphonates
  • vitamin D
  • calcium
  • folic acid

Surgery

Your doctor may suggest surgical removal of the spleen if the steroids don’t work completely. Removal of the spleen can reverse the destruction of red blood cells. This surgery is known as a splenectomy. Two-thirds Trusted Source of people who undergo a splenectomy have a partial or total remission from their AIHA, and people with the idiopathic type tend to have the most successful results.

Immune-suppressing drugs

Other treatment options are immune-suppressing drugs, such as azathioprine and cyclophosphamide. These can be effective medications for people who don’t successfully respond to treatment with steroids or who aren’t candidates for surgery.

In some cases, the medication rituximab may be preferred over the traditional immune-suppressing drugs. Rituximab is an antibody that directly attacks specific proteins found on certain immune system cells.

Long-term outlook

It can be difficult to get a quick diagnosis of this condition in cases where the cause is unknown. Treatment is sometimes delayed in these cases. Idiopathic AIHA can be fatal if left untreated.

Idiopathic AIHA in children is typically short-lived. The condition is often chronic in adults and can flare up or reverse itself without explanation. AIHA is highly treatable in both adults and children. Most people make a full recovery.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.

Medication

A doctor may prescribe corticosteroids or cortisone-like drugs to weaken the immune response.

This is generally 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 own 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.

Surgery

If drug treatment is not effective, a doctor may recommend surgery.

The spleen is responsible for removing abnormal red blood cells from the bloodstream, including those with antibodies attached. Removing the spleen can enable the body to preserve those red blood cells. This can help to prevent anemia.

Blood transfusion

If symptoms are severe and other options are not effective, the person may need a blood transfusion.

AIHA in children

AIHA can occur in children. However, 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 pre-school age children.

When AIHA occurs in children, it is usually the result of a virus or infection.

Often no treatment is necessary, and symptoms will pass without intervention. Children who need treatment will have the same treatment options as adults.

AIHA can disrupt a child’s everyday routine due to tiredness and the need for ongoing medical support, including tests.

Parents and caretakers should ensure that the child:

  • follows a well-balanced diet
  • gets plenty of rest and fluids
  • plans activities in a way that will enable the child to manage their condition

A doctor will discuss a specific treatment plan.

Prevention

It is not possible to prevent some types of AIHA, but doctors can monitor people who have a viral infection or who use certain medications, to ensure that AIHA does not develop.

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.

Lifestyle management

If someone has AIHA, their doctor will work with them to help reduce symptoms and the chance of complications.

  • avoiding people with infections or who are sick
  • washing hands and brushing teeth regularly to reduce the risk of oral and other infections
  • having an annual flu shot

People with cold AIHA should try to keep warm, as a cold environment can trigger the breakdown of red blood cells.

Takeaway

The outlook for AIHA is usually good. The condition usually lasts for a limited time.

If it occurs during the teenage years, it can become a long-term condition. However, medical treatment can help reduce the impact.

 

 

 

 

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