Autoimmune hemolytic anemia is a specific form of anemia that is acquired rather than congenital. The condition is also referred to in medical parlance by the abbreviation AIHA. The disease is triggered by certain antibodies directed against the body’s own antigens. The antibodies bind primarily to the red blood cells (scientific term erythrocytes) and in some cases destroy these blood cells. This destruction process is also called hemolysis.
What is autoimmune hemolytic anemia?
Basically, different forms of autoimmune hemolytic anemia exist. Essentially, it is differentiated into four distinct categories. The differences between these forms lie primarily in the various causes for the development of autoimmune hemolytic anemia. Thus, there is autoimmune hemolytic anemia of the Donath-Landsteiner type, the heat type, the cold type, and AIHA caused by certain drugs. A characteristic feature of all manifestations of the disease is that the immune system produces antibodies that lead to the destruction of red blood cells. Autoimmune hemolytic anemia of the heat type is the most common. Approximately three quarters of all cases of the disease are in this category. In this case, so-called heat antibodies are formed by the immune system. In second place is cold-type AIHA, which accounts for about one fifth of all diseases. Far less common are autoimmune hemolytic anemias associated with bithermic antibodies. In addition, a distinction is made between complete and incomplete antibodies. The former cause a clearly visible clumping of the blood (medical term agglutination). This causes the red blood cells to stick together. In the presence of incomplete antibodies, no clumping of the blood cells occurs. The complete antibodies usually 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 detected with a so-called direct Coombs test. Antibodies within the blood serum are detectable by indirect Coombs testing.
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 proliferation at body temperature. They are often so-called immunoglobulins G, much more rarely A or M. In principle, the disease manifests itself at any age, but adults are most frequently affected. In addition, female patients suffer from the disease more often than males. In approximately 50 percent of all cases, external causes for the development of autoimmune hemolytic anemia cannot be determined. In the remaining cases, links to various external agents or infections are evident. Very rarely, for example, vaccinations are responsible for the disease. Other potential triggers are immunodeficiencies, malignant lymphomas, leukemia, thymomas or collagenoses. Other causes are present in autoimmune hemolytic anemia of the cold type. When body temperature drops, cold agglutinins find optimal conditions. These are usually immunoglobulins M. They cause red blood cells to combine. Subsequently, destruction of the cells occurs. Potential causes of this process include malignant lymphoma, a monoclonal gammopathy such as Waldenström’s disease, or various infections.
Symptoms, complaints, and signs
Autoimmune hemolytic anemia is characterized by a number of symptoms. Typical signs of anemia include fatigue, pale skin, low performance, tachycardia, and shortness of breath on physical exertion. In addition, typical symptoms of hemolysis appear. These include, for example, urine of dark 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 is highly dependent on the individual case. If hemolysis is severe, affected patients suffer from symptoms such as chills, nausea and vomiting, abdominal pain, and fever. This condition may pose an acute threat to the life of the ill person.If hemolysis is extreme, shock and renal failure are possible. When drugs are the triggers for autoimmune hemolytic anemia, symptoms usually present a few minutes to hours after administration. In cold-type autoimmune hemolytic anemia, intravascular hemolysis occurs.
Diagnosis and course
If the presence of autoimmune hemolytic anemia is suspected on the basis of typical symptoms, a physician should be consulted urgently. The medical history already provides relevant information about the disease. In order to diagnose autoimmune hemolytic anemia with certainty, blood tests are primarily helpful. The type of antibody present in each case is identified. Particularly significant at this point is the performance of the Coombs test. In autoimmune hemolytic anemia, the direct Cooms test is usually positive. If heat antibodies are seen, the patient’s medications should be checked. Lymphoma should also be ruled out. When mixed antibodies are detected, it is sometimes necessary to first cool and then rewarm the blood sample.
Complications
In most cases, this anemia causes marked fatigue and headache in the patient. Performance decreases sharply and it is no longer possible for the patient to perform physically strenuous work and activities. Likewise, most patients suffer from pale skin and shortness of breath. The shortness of breath can also lead to a panic attack in many patients. The quality of life is greatly reduced by the symptoms. Psychological complaints and depression can also develop. Often, the symptoms of a common flu also occur, such as chills, diarrhea and vomiting. It is not uncommon for fever and abdominal pain to occur as well. In the worst case, this anemia leads to kidney failure, in which case it is a life-threatening condition for the patient. Treatment is usually causal and depends on the underlying disease of the anemia. Often, the patient is administered medications that abate the symptoms and relieve the discomfort. With successful and early treatment, no further complications occur. If the kidneys have been damaged, the affected person may be dependent on dialysis.
When should you see a doctor?
Autoimmune hemolytic anemia (AIHA) occurs in three variants, depending on whether the autoimmune hemolysis is due to heat antibodies, cold antibodies, or a mixed form. Common to all three courses is that the antibodies – usually type G immunoglobulins (IgG) – dock onto the receptors of red blood cells (erythrocytes), thus “releasing” them for destruction by the immune system via hemolysis. It is advisable to seek medical advice and have a blood test performed for specific antibodies at the first symptoms that may indicate AIHA disease. Signs of the disease can be quite nonspecific such as fatigue, decreased performance, shortness of breath and chills, fever and nausea. In cases of severe hemolysis, there is bilirubin accumulation, which darkens the urine and makes the skin appear yellowish. Above all, the physician must clarify differential diagnosis to rule out other diseases with similar symptoms, such as lymphoma or infection with mycoplasma. If acute AIHA is caused by heat antibodies, it must also be clarified whether medications taken, e.g., an antibiotic related to penicillin or methyldopa for lowering blood pressure, may be the trigger of the disease. In about half of all cases of autoimmune hemolytic anemia, the causative agent cannot be determined, and the disease is then idiopathic AIHA.
Treatment and 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 given.
Outlook and prognosis
With timely medical treatment, there is a good prognosis in autoimmune hemolytic anemia. Nevertheless, it depends on the underlying disease present, the age of the patient, and the patient’s health status.Adults who do not have any previous illnesses and who have a healthy immune system have the best chances of recovery. They can be discharged as healthy and symptom-free after a few weeks or months of treatment. For follow-up care, they will continue to receive medical care until the body has regained full health. If there are pre-existing conditions, the prognosis outlook must be considered in conjunction with the conditions. In chronic diseases, the immune system is usually weakened. If the patient suffers from severe diseases, the prospect of recovery is also significantly diminished. In the case of diseases affecting the blood system, the prognosis prospects drop even further. Nevertheless, these patients have far better prospects of recovery than people without medical care. Without medical treatment, life-threatening sequelae occur. Initially, there are everyday limitations due to a drop in performance and persistent fatigue. As the disease progressively spreads, respiratory distress, heart problems and anxiety may develop in the subsequent course. 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 possible only by preventing the underlying infections or diseases.
Follow-up
There are very limited or no measures or options available to the affected person for direct aftercare in this disease. First and foremost, this disease requires early diagnosis with subsequent treatment to prevent further complications or discomfort. A medical doctor must be contacted at the first symptoms of this anemia so that the symptoms are not exacerbated. Possibly the life expectancy of the affected person is reduced by this disease. It is also not possible to cure the disease on one’s own. The treatment itself is usually carried out by taking medication. When taking this medication, the affected person should in any case ensure that it is taken regularly and in the correct dosage, so that the symptoms are permanently alleviated. Regular checks and examinations by a doctor are also very useful and necessary. Furthermore, the underlying disease must also be recognized and treated in order to permanently reduce this complaint. However, no general course of the disease can be given. Not infrequently, however, those affected are dependent on the help and support of their own family.
This is what you can do yourself
Autoimmune hemolytic anemia has three variants, but its course is the same with respect to the destruction of the immune system via hemolysis. Provided the underlying cause has been medically clarified, affected individuals can successfully support the treatment plan with some self-help measures in everyday life. Bad lifestyle habits such as smoking, excessive alcohol consumption and drug abuse must be discontinued. The daily diet should include at least three liters of liquid and foods that contain a lot of iron, such as spinach, beet, cress, parsley, especially in combination with lemon, which optimally processes the iron, and linseed oil as an omega-3 fatty acid carrier. Fat-rich contentless products and too much of caffeinated drinks have a destructive effect. Partly, the symptom can be traced back to drug abuse. Therefore, it is important to get detailed advice in the context of a therapy. Accordingly, accompanying states of exhaustion, permanent fatigue and headaches can be adequately treated. Extensive walks in nature as well as Tai-Chi are recommended. Stress and physically heavy work should be avoided. If conditions of shortness of breath and acute kidney pain occur, they should be reported to emergency medical services and not self-medicated. Blood transfusions may alleviate autoimmune hemolytic anemia. For those affected by the symptom who are on dialysis, thorough personal hygiene and a low-salt diet should be a priority.
