When autoimmune hemolytic anemia is a special form of anemia that is not innate, but acquired. In medical colloquial language, the disease is also referred to by the abbreviation AIHA. The disease is triggered by certain antibodies that are directed against the body’s own antigens. The antibodies mainly bind to the red blood cells (scientific name erythrocytes) and in some cases destroy these blood cells. This destruction process is also called hemolysis.
What is Autoimmune Hemolytic Anemia?
Basically there are different forms of autoimmune hemolytic anemia. There are essentially four different categories. The differences between these forms lie primarily in the various causes for the development of autoimmune hemolytic anemia.
There is an autoimmune hemolytic anemia of the Donath-Landsteiner type, the warm type, the cold type and an AIHA, which is caused by certain drugs. A characteristic of all forms of the disease is that the immune system produces antibodies that lead to the destruction of red blood cells.
The most common is autoimmune hemolytic anemia of the warm type. Around three quarters of all cases of illness fall into this category. So-called heat antibodies are formed by the immune system. In second place is the AIHA of the cold type, which makes up about a fifth of all diseases. Autoimmune hemolytic anemias associated with bithermal antibodies are far less common.
In addition, a distinction is made between complete and incomplete antibodies. The former cause a clearly visible clumping of the blood (medical term agglutination). The red blood cells stick together. If incomplete antibodies are present, no clumping of the blood cells occurs.
The complete antibodies mostly belong to the immunoglobulins M, the incomplete ones are immunoglobulins G. If the antibodies are on the surface of the red blood cells, they can be determined with a so-called direct Coombs test. Antibodies within the blood serum can be detected using indirect Coombs tests.
Causes
The causes of autoimmune hemolytic anemia depend on the type of disease. The most common is autoimmune hemolytic anemia of the heat type. The responsible antibodies reach optimal conditions for spreading at body temperature.
Often these are so-called immunoglobulins G, far less often A or M. In principle, the disease occurs at any age, but adults are most often affected. In addition, female patients suffer from the disease more often than males. In about 50 percent of all cases of illness, external reasons for the development of autoimmune hemolytic anemia cannot be determined.
In the remaining cases there are connections to various external influences or infections. Vaccinations, for example, are very rarely responsible for the disease. Other potential triggers are immune defects, malignant lymphomas, leukemia, thymomas or collagenoses.
Other causes are cold-type autoimmune hemolytic anemia. If the body temperature drops, cold agglutinins find optimal conditions. These are usually immunoglobulins M. They cause the red blood cells to combine.
The cells are then destroyed. Potential causes for this process are, for example, malignant lymphomas, monoclonal gammopathy such as Waldenström’s disease or various infections.
Symptoms, ailments & signs
Abbreviated as AIHA by ABBREVIATIONFINDER.ORG, autoimmune hemolytic anemia is characterized by a number of complaints. Signs of anemia such as tiredness, pale skin, poor performance, tachycardia and shortness of breath during physical exertion are typical. In addition, typical symptoms of hemolysis appear.
These include, for example, urine that is dark in color due to the release of the blood pigment hemoglobin and a yellowish discoloration of the skin due to an accumulation of bilirubin. Basically, the clinical progression of the disease depends heavily on the individual case.
If the hemolysis is severe, the affected patients suffer from symptoms such as chills, nausea and vomiting, abdominal pain, and fever. Under certain circumstances, this condition represents an acute threat to the life of the sick person. If the hemolysis is extreme, shock and failure of kidney function are possible.
When drugs cause autoimmune hemolytic anemia, symptoms usually appear a few minutes to hours after administration. In cold-type autoimmune hemolytic anemia, intravascular hemolysis occurs.
Diagnosis & course
If autoimmune hemolytic anemia is suspected based on typical symptoms, a doctor should be consulted urgently. The anamnesis already gives relevant information about the disease. Blood tests are primarily helpful in diagnosing autoimmune hemolytic anemia with certainty.
The type of antibody present is identified in each case. Carrying out the Coombs test is particularly important at this point. In autoimmune hemolytic anemia, the direct Cooms test is usually positive. If heat antibodies are found, the patient’s drugs should be checked.
Lymphomas can also be ruled out. If mixed antibodies are detected, it is sometimes necessary to cool the blood sample first and then to warm it up again.
Complications
In most cases, this anemia results in pronounced fatigue and headaches in the patient. The performance decreases sharply and it is no longer possible for the patient to perform physically strenuous work and activities. Most patients also suffer from pale skin and shortness of breath.
The shortness of breath can also lead to a panic attack in many patients. The symptoms severely limit the quality of life. Psychological complaints and depression can also develop. Common flu symptoms such as chills, diarrhea, and vomiting are also common. It is not uncommon for a fever and abdominal pain to occur.
In the worst case, this anemia leads to kidney failure, which is a life-threatening condition for the patient. The treatment is usually causal and depends on the underlying disease of anemia. Medication is often given to the patient to ease the symptoms and alleviate the discomfort.
With successful and early treatment, no further complications arise. If the kidneys have been damaged, they may need dialysis.
When should you go to the doctor?
Autoimmune hemolytic anemia (AIHA) occurs in three variants, depending on whether autoimmune hemolysis is based on heat antibodies, cold antibodies or a mixed form. All three forms of progression have in common that the antibodies – mostly immunoglobulins of type G (IgG) – dock on the receptors of the red blood cells (erythrocytes) and thus “release” them for destruction by the immune system via hemolysis. It is advisable to seek medical advice at the first symptoms that could indicate an AIHA disease and to have a blood test carried out for certain antibodies.
Signs of the disease can be quite unspecific, such as exhaustion, reduced performance, shortness of breath and chills, fever and nausea. With severe hemolysis, bilirubin accumulates, which turns the urine dark and makes the skin appear yellowish. Above all, the doctor must use differential diagnosis to clarify whether other diseases with similar symptoms, such as lymphoma or mycoplasma infection, can be ruled out.
If the acute AIHA is caused by heat antibodies, it must also be clarified whether the medication taken, e.g. For example, a penicillin-related antibiotic or methyldopa, used to lower blood pressure, may be the cause of the disease. In about half of all cases of autoimmune hemolytic anemia, the cause cannot be determined, so that it is then an idiopathic AIHA.
Treatment & Therapy
Autoimmune hemolytic anemia is treated with blood transfusions. Basically, it is important to treat the cause of the disease. If this is not possible, immunosuppression is used. In addition, the drugs cyclophosphamide and rituximab are sometimes administered.
Outlook & forecast
With timely medical treatment, autoimmune hemolytic anemia has a good prognosis. However, it depends on the underlying disease, the age of the patient and their state of health.
Adults who have no previous illnesses and who have a healthy immune system have the best chance of recovery. These can be discharged as healthy and symptom-free after a few weeks or months of treatment. For aftercare, they will continue to receive medical care until the body has regained its full health.
If there are previous illnesses, the prognosis perspective must be considered in connection with the illnesses. In chronic illnesses, the immune system is usually weakened. If the patient suffers from serious illnesses, the prospect of a cure is also significantly reduced. In the case of diseases that affect the blood system, the prognosis continues to decline. Yet these patients have far better prospects of recovery than people without medical care.
Without medical treatment, life-threatening secondary diseases can occur. Initially, there are everyday restrictions due to a drop in performance and persistent fatigue. As the disease spreads gradually, it can lead to shortness of breath, heart problems and anxiety.
In the most severe case, kidney failure or heart attack occurs. The patient is threatened with lifelong impairments and functional disorders. In addition, the risk of premature death increases.
Prevention
Prevention of autoimmune hemolytic anemia is only possible by preventing the underlying infections or diseases.
Aftercare
With this disease, the person affected has only very limited or no measures or options for direct follow-up care available. First and foremost, this disease must be diagnosed early and then treated so that there are no further complications or complaints. A doctor has to be contacted as soon as the first symptoms of this anemia appear, so that the symptoms do not worsen.
This disease may reduce the life expectancy of the person affected. In doing so, there cannot be an independent healing. The treatment itself is usually carried out by taking medication. With this intake, the person concerned should make sure to take it regularly with the correct dosage so that the symptoms are permanently alleviated.
Regular checks and examinations by a doctor are also very useful and necessary. In the case of this anemia, however, the underlying disease must also be identified and treated in order to limit this complaint permanently. However, no general course of the disease can be given. However, it is not uncommon for those affected to rely on the help and support of their own families.
You can do that yourself
Autoimmune hemolytic anemia has three variants, but its course is the same with regard to the destruction of the immune system by hemolysis. If the underlying cause has been clarified medically, those affected can successfully support the therapy plan with some self-help measures in everyday life.
Bad habits such as smoking, excessive alcohol and substance abuse should be abandoned. The daily nutrition plan includes at least three liters of liquid and foods that contain a lot of iron, such as: spinach, beetroot, cress, parsley, especially in combination with lemon, which optimally processes the iron, and linseed oil as an omega-3 fatty acid carrier. High-fat, contentless products and too much caffeinated drinks have a destructive effect.
In part, the symptom can be traced back to drug abuse. It is therefore important to seek detailed advice as part of a therapy. Accordingly, accompanying states of exhaustion, long-term fatigue and headaches can be adequately treated. Extensive walks in nature and tai chi are recommended. Avoid stress and hard physical work.
If conditions of shortness of breath and acute kidney pain occur, these must be reported to the medical emergency service and not treated yourself. Blood transfusions can reduce autoimmune hemolytic anemia. For those affected who are bound to dialysis due to the symptom, thorough personal hygiene and a low-salt diet should be the focus.
