The term acute transient erythroblastopenia is used to describe a temporary poverty of erythroblasts, the precursor cells of erythrocytes. The disease causes transient anemia for reasons that are often unknown, because the process of red blood cell formation (erythropoiesis) from bone marrow stem cells is temporarily slowed or interrupted. In infants and young children, it is the most common form of anemia that is not due to altered red blood cells.
What is acute transient erythroblastopenia?
Due to the decreased number of erythroblasts, acute transient erythroblastopenia results in anemia that is based solely on an insufficient number of erythrocytes. Acute transient erythroblastopenia is a transient decreased concentration of erythroblasts. Erythroblasts are formed by cell division and differentiation from macroblasts within erythropoiesis. Erythropoiesis is the overall process of red blood cell (erythrocyte) formation from multipotent stem cells of the bone marrow. Decreased number of erythroblasts results in anemia, which is solely due to insufficient number of erythrocytes and not due to iron deficiency or erythrocytes with functional deficiencies. Transient erythroblastopenia causes the form of anemia that is the most common form of normocytic anemia in infants and young children. Normocytic in this context means that the erythrocytes present are of normal size and normal functionality. Essential in the transient form of erythroblastopenia is its transient manifestation. Normally, the interruption or inhibition of erythropoiesis lasts about one to two weeks. Thereafter, there is a spontaneous recovery of erythropoiesis with regeneration and resolution of the anemic state.
Causes
Frequently, diagnosed acute transient erythroblastopenia is termed idiopathic, indicating that the causes that triggered the disease are unknown. What appears to be certain is that certain substances cause a transient interruption of erythropoiesis lasting approximately one to two weeks. Certain drugs as well as viral infections are considered as causative agents of acute transient erythroblastopenia. For example, some authors link the causative agent of ringworm, parvovirus B19, to transient erythroblastopenia. Ringworm is one of the classic childhood diseases and is usually harmless. Chronic hemolysis, in which ongoing dissolution of the cell membrane of erythrocytes occurs and hemoglobin leaks into the plasma, is also thought to be a possible causative agent of acute transient erythroblastopenia.
Symptoms, complaints, and signs
Initial signs and symptoms of acute transient erythroblastopenia are quite nonspecific and very similar to those anemias acquired for other reasons. Noticeable features include a translucent pallor of the skin, rapid onset of fatigue, and a general decrease in performance. The general symptoms are usually accompanied by headache. The symptoms may ultimately be attributed to decreased oxygen and nutrient supply to the brain, muscle tissues, and other tissues requiring oxygen. If the disease is pronounced, there is also difficulty breathing (dyspnea) and an accelerated pulse (tachycardia). Acute transient erythroblastopenia shows no signs of inflammation. For example, there is no swelling of the spleen, liver, or lymph nodes, which might otherwise indicate inflammatory reactions.
Diagnosis and course
In addition to the nonspecific symptoms and signs of acute transient erythroblastopenia described above, an examination of the blood count provides a clear diagnosis. A normocytic anemia is seen, meaning that the red cells present are within the tolerable range in terms of size, i.e., they show no abnormalities. However, the reduced number of reticulocytes, which represent the young, not yet fully differentiated, erythrocytes within the chain of erythropoiesis, is conspicuous. In some cases, myelopoiesis is also impaired, i.e. the process that, in addition to erythropoiesis, also involves the formation of various white blood cells such as granulocytes, monocytes and platelets in the bone marrow.This means that the blood count may also show a reduced number of neutrophil granulocytes, for example. The course of the disease is normally self-regulating. Normally, erythropoiesis regenerates within one to two weeks, so that the symptoms also subside without therapy. If this is not the case, an incorrect diagnosis has been made.
Complications
Acute transient erythroblastopenia is defined as a temporary arrest of red blood cell production. This type of anemia is predominantly seen in infants and young children. Since the process is inhibited directly within the bone marrow stem cells, affected individuals belong in medical treatment to be on the safe side. Ringworm pathogens, infections, various substances and drugs, as well as chronic hemolysis are suspected as triggers of the symptom. These then lead to an interruption of red blood cell formation for about five to fourteen days. During this time, the signs can cause some complications to the affected person. In addition to the noticeable pallor, severe episodes of fatigue accompanied by severe headaches and a considerable reduction in performance are also evident. If the initial features of acute transient erythroblastopenia are ignored, this can lead to shortness of breath and palpitations even at rest. Especially in infants, there is a risk of undersupply of muscle tissue and brain due to low oxygen and nutrient supply to the blood. In most cases, with sufficient rest and good nutrition, the patient regenerates on his or her own without the need for medication. If the disease is too advanced, heart failure may develop as a complication risk. In the case of a blood transfusion, an immediate measure is to provide the affected person with sufficient red blood cells to eliminate the risk of cardiovascular failure.
When should you go to the doctor?
If acute transient erythroblastopenia or a similar condition is suspected, the patient should see a doctor immediately. If symptoms such as headache, fatigue, and a general decrease in performance are observed, this must be discussed with a physician. Externally, the condition is manifested by pale skin and dark circles under the eyes, among other symptoms. If one of these symptoms is noticed, the rule is: go to the doctor and have the cause determined. Although the symptoms do not necessarily indicate acute transient erythroblastopenia, they do indicate a condition that should be clarified and treated. If anemia is left untreated, it can lead to shortness of breath or palpitations in the further course. Infants are particularly at risk, and should be taken to a pediatrician at the first sign of anemia. Adults should consult a doctor if the symptoms do not subside on their own despite sufficient rest and good nutrition. In advanced stages, cardiovascular problems may occur and should always be treated by an emergency physician.
Treatment and therapy
Because acute transient erythroblastopenia is usually transient in nature, many cases do not require therapy. Spontaneous regeneration of erythropoiesis can be supported and accelerated by taking erythropoietin, iron, and folic acid. The polypeptide erythropoietin, which is composed of 165 amino acids, is a glycoprotein hormone and belongs to the group of cytokines. The hormone intervenes in the process of erythropoiesis in a controlling manner. Erythropoietin is also known as a doping agent under the infamous name EPO. Immediate action is only required in cases of severe disease, where there is a risk of heart failure and cardiogenic shock. Because of the lack of differentiated erythrocytes, which cannot be readily corrected by drugs or other measures, the most effective treatment option in these cases is a blood transfusion, which provides the necessary supply of erythrocytes, at least temporarily.
Outlook and prognosis
Transient erythroblastopenia directly causes anemia in the patient in most cases. In this case, the affected person appears tired and weary and no longer actively participates in life. Severe headaches occur, which can also spread to other parts of the body. If the pain also occurs at night, transient erythroblastopenia can lead to sleep disturbances. Usually, there is also a reduced supply of oxygen, so that the extremities and organs are undersupplied.In the worst case, this can lead to the death of individual organs or extremities, after which death can sometimes occur. Furthermore, infections can occur in the liver or spleen. The quality of life of the affected person is extremely reduced by the disease. The ability to cope with stress also decreases enormously, so that the patient can no longer carry out normal everyday activities or sporting activities without further ado. Treatment is carried out with the help of medication and can limit the symptoms. In severe cases, transfusions are necessary. Furthermore, damage to the organs or extremities may need to be treated.
Prevention
Because the etiology of acute transient erythroblastopenia is not adequately known, directly preventive measures to counteract the onset of the disease are nonexistent. Nor is it known whether certain genetic predispositions are among the risk factors. Since the disease mostly occurs in infants and young children, measures that strengthen the immune system can be considered favorable in terms of preventive measures. It is not possible to say with certainty whether this can effectively prevent the occurrence of the disease. It is recommended that when the transient form of erythroblastopenia is suspected, differential diagnosis should be used to rule out other diseases that may require immediate treatment.
Follow-up
In this disease, the affected person is primarily dependent on rapid and, above all, early detection and treatment, so that further complications or complaints do not occur. The earlier a medical professional is consulted in this regard, the better the further course of this disease usually is. It cannot be universally predicted whether this disease will reduce the life expectancy of the person affected. However, there are usually no special measures or options for aftercare available to the affected person, and self-healing cannot occur either. The focus is therefore on early detection and treatment of this disease. In the treatment of this disease, the affected person is primarily dependent on taking various medications. It is important to pay attention to a correct dosage and also to a regular intake in order to counteract the symptoms. Since this disease can also cause various heart problems, regular cardiac examinations should be performed. The affected person should not put unnecessary strain on his body and should also pay attention to a healthy diet and a healthy lifestyle. In some circumstances, contact with other sufferers of the disease may also be useful in this regard.
What you can do yourself
Acute transient erythroblastopenia usually does not require special treatment. Adults are affected, but primarily young children. Symptoms may occur in addition to the characteristic pallor, such as headache, decreased performance, and faintness. Nevertheless, some things can be done to improve the well-being. Fresh air, physical rest and moderate exercise do good. However, excessive demands and great exertion should be avoided. Parents of affected children can ensure that rest periods are observed. Rest is also good for affected adults. Cold compresses can be offered for headaches, and in more severe cases, pain medication prescribed by a doctor. Quiet activities, reading aloud and listening to radio plays are beneficial for the sick child. Adult patients also avoid overloading themselves with intense physical activity. Furthermore, attention should be paid to a healthy diet with fresh fruits, vegetables, meat and fish. The disease regularly improves without further intervention. However, it makes sense to have regular medical check-ups and to observe the symptoms. Even if the disease requires special medical therapy only in exceptional cases, watch out for signs of shock or possible heart failure. If such signs are present, the attending physician should be consulted. Acute transient erythroblastopenia usually heals within two weeks.
