When erythremia is a specific expression of myeloid leukemia with acute course. Basically about five percent of all leukemias represent erythremia. There is both a chronic and an acute type of erythremia. In earlier times, polycythemia vera was also considered erythremia.
What is erythremia?
According to sciencedict.com, erythremia is also known by the synonymous terms erythremic myelosis and erythroleukemia. Usually erythremia is an acute form of myeloid leukemia. Erythremia develops as a result of degenerate manufacturing processes of erythrocytes.
In medicine, this process is also known as erythropoiesis. Histological examinations of patients with erythremia show a majority of immature erythrocyte cell types, the so-called erythroblasts. Due to the increased volume of the cells, doctors also call these types megaloblasts.
Causes
The way in which erythremia develops in the affected people is currently still the subject of medical research. Because the exact processes involved in the pathogenesis of erythremia are not yet exactly known at the present time. Basically, the processes involved in erythropoiesis are degenerate, so that erythremia develops.
Erythropoiesis is the process of making red blood cells. This results in an increased number of immature erythrocytes, which only live for a relatively short time compared to other blood cells. Doctors call these components of the blood erythroblasts. If these blood cells are unusually large, they are called megaloblasts.
In the context of erythremia, there are considerable differences in the size of the red blood cells and their precursor types. In addition, the pathological blood cells are characterized by an abnormal shape compared to healthy blood components.
Symptoms, ailments & signs
Erythremia manifests itself in the sick patient in a number of characteristic signs of illness and pathological complaints. The main symptoms of erythremia are an increased body temperature and an enlarged liver and spleen. Since people affected by erythremia suffer from a lack of platelets, the tendency to bleed increases, for example with minor injuries or inside the body.
As a result, erythremia is usually accompanied by severe anemia. This also increases the risk of developing infectious diseases for those suffering from erythremia. Both the spleen and the liver swell significantly in the course of erythremia and are typical features of acute erythremia.
The reduced number of granulocytes in the blood also increases the likelihood of developing infections for patients with erythremia. If there is no adequate and timely therapy for erythremia, the affected people often die after a few months as a result of the symptoms of the disease and the associated complications.
Chronic erythremia occurs much less frequently than the acute form. Unlike the acute type of erythremia, this disease is often much milder. Sometimes it is difficult to distinguish it from sideroblastic anemia, because both diseases have a disruption of iron utilization. In chronic erythremia, however, the erythroblasts are less subject to anomalies.
Diagnosis
The first signs of erythremia prompt the patient to consult a doctor immediately. In the case of existing erythremia, it should always be borne in mind that the disease is often fatal for those affected without adequate treatment. A general practitioner records the symptoms for the first time and usually refers the patient to a hematologist or a comparable specialist.
When taking the anamnesis, the treating specialist identifies the symptoms and asks about the beginning of the symptoms. The further procedure for diagnosing erythremia is largely similar to the procedures that are also used for other forms of leukemia. In the clinical examination of erythremia, the focus is primarily on a puncture of the bone marrow and various analyzes of the blood.
The diagnosis of erythremia is largely based on the results of the bone marrow aspiration. In erythropoiesis, there is a shift to the left. The differential diagnosis of erythremia is often difficult, although the doctor has to differentiate the disease from other subtypes of leukemia.
Complications
As a rule, erythremia leads to a greatly increased body temperature. This is also associated with enlargement of the liver and spleen, which can lead to pain in these areas. The affected person suffers from an increased risk of bleeding. Even minor injuries can lead to profuse bleeding, which can also spread inside.
The person concerned also suffers from an increased risk of infectious diseases. If the erythremia is not treated properly or not treated quickly enough, death usually results. The treatment itself is carried out with the help of medication and supported by blood transfusions. The patient has to be prepared for a long stay in a hospital.
In rare cases, complications arise from the blood transfusion. To slow down cancer growth, people are also given cytotoxic drugs. In most cases, erythremia can be limited and treated completely with early treatment.
However, the patient must have regular medical check-ups. The treatment does not rule out the possibility of the erythremia recurring later in life. Proper treatment will not reduce life expectancy.
When should you go to the doctor?
Unfortunately, in many cases the symptoms of erythremia are not particularly characteristic and do not point directly to the disease. However, an early diagnosis increases the chances of a complete cure of the disease.
A doctor should be consulted if the person concerned has been suffering from an elevated body temperature for a long period of time. The high tendency to bleed can also indicate erythremia. The symptoms of the disease also include an enlarged spleen and liver, which in some cases can lead to pain.
Anemia also sets in, which can lead to permanent tiredness and fatigue in the patient. If these symptoms occur very frequently and without a particular reason, an examination by a doctor is always necessary. Usually, the erythremia can be diagnosed directly by a general practitioner. A pediatrician can also be consulted for children. Further treatment then depends on the respective underlying disease, so that the help of other specialists is necessary.
Treatment & Therapy
Treatment for erythremia begins as soon as possible after the diagnosis is made. The therapeutic measures usually take place in a specialized center. The treatment of erythremia focuses on blood transfusions and drug therapy approaches. The transfusions compensate for the shifts in the concentration of the various blood cells and also compensate for the pathological changes in the red blood components.
Since the effects of blood transfusions in erythremia only last a limited time, several transfusions are usually required at fixed intervals. At the same time, the patients receive cytostatics. These are medicinal substances that slow the growth of tumors. Continuous patient care is important in order to check the success of the treatment measures and to improve the prognosis of erythremia.
Outlook & forecast
Without treatment, erythremia is incurable and always leads to death. If left untreated, acute erythremia ends fatally after a few months. In the case of chronic erythremia, there is an average life expectancy of around two years under these conditions. However, good results in extending life expectancy can be achieved with treatment.
Therapy mainly consists of the use of cytotoxic drugs and blood transfusions. Cytostatics inhibit the growth of cancer cells. Complete remission of the cancer cells can be achieved in around 70 percent of patients. However, the older the patient, the worse they respond to chemotherapy with cytostatics.
Studies have shown that in those over 60 years of age, the rate of complete remission is only between 30 and 60 percent. However, complete remission does not necessarily mean that there is a complete cure. Blood tests only show that cancer cells are no longer detectable. However, in most patients these are not completely killed, so there is a high relapse rate. Only 15 to 25 percent of all patients achieve long-term remission so that they can be considered cured. It is not yet clear whether these are real cures.
Complete healing can only be achieved through a stem cell transplant. However, this is only carried out in exceptional cases in younger patients or in patients with poor responsiveness to chemotherapy. A stem cell transplant carries the risk of serious side effects from immune reactions.
Prevention
Concrete possibilities for an effective prevention of erythremia have not been tried out. Although certain mechanisms of the pathogenesis of erythremia have already been researched, they are largely beyond the influence of medicine.
Aftercare
In the case of erythremia, in most cases there are only very limited options for follow-up care. The patient is primarily dependent on medical treatment by a doctor so that there are no further compilations. Early diagnosis with early treatment is particularly important in order to properly treat the disease.
Since those affected are dependent on transfusions due to erythremia, these should be carried out regularly. Furthermore, infections and other diseases are to be avoided as far as possible in order not to weaken and burden the immune system even more. Since erythremia can also contribute to the development of tumors, regular examinations should be carried out in order to detect and treat them at an early stage.
The patient’s life expectancy may also be limited by the disease. Contact with other sufferers of the disease can also be useful and have a positive effect on the further course. In the case of mental disorders, intensive and loving care by the parents is very important in order to alleviate these complaints.
You can do that yourself
If erythremia is suspected, those affected should consult a doctor immediately. Under no circumstances should you try to treat the disease yourself. Acute erythremia can lead to death within a few months. The most important self-help measure is therefore to recognize the symptoms and seek professional medical help promptly.
Typical of the disease are an increased body temperature combined with an enlarged liver and spleen. The organ enlargements are usually imperceptible to those affected. However, due to a lack of platelets, the disease is also associated with greatly impaired wound healing and pronounced anemia. Anyone who constantly feels tired and exhausted, is very pale and also notices that even small injuries bleed heavily and for a long time, should definitely consult a doctor immediately.
The patients are usually treated with blood transfusions and the administration of cytostatics. In the naturopathy natural cytostatic agents such as amygdalin are often used stone fruit seeds. The effectiveness of such agents is questionable. Their use, after consulting the attending physician, but usually harmless. Other measures recommended in naturopathy for leukemia, such as a change in diet, can improve general well-being and support the treatment of the disease.