Erythremia is a particular manifestation of myeloid leukemia with an acute course. Basically, about five percent of all leukemias represent erythremias. There is both a chronic and an acute type of erythremia. In earlier times, polycythaemia vera was also considered an erythremia.
What is erythremia?
Erythremia is also known by the synonymous terms erythremic myelosis and erythroleukemia. In most cases, erythremia is an acute form of myeloid leukemia. Erythremia develops as a result of degenerate manufacturing processes of erythrocytes. This process is also known in medicine as erythropoiesis. Histological examinations of patients with erythropoiesis show an excess of immature erythrocytic cell types, the so-called erythroblasts. Due to the increased volume fraction of the cells, physicians also refer to these types as megaloblasts.
Causes
The way in which erythremia develops in affected individuals is currently still the subject of medical research. This is because the exact processes involved in the pathogenesis of erythremia are not precisely known at the present time. Basically, the processes within erythropoiesis are degenerated so that erythremia develops. Erythropoiesis is the process of red blood cell production. During this process, an increased number of immature erythrocytes are produced, which have a relatively short life span compared to other blood cells. Physicians call these components of the blood erythroblasts. If these blood cells are unusually large, they are known as megaloblasts. In the context of erythroemia, significant differences in the size of red blood cells and their precursor types become apparent. In addition, the pathologic blood cells are characterized by an abnormal shape compared with healthy blood components.
Symptoms, complaints, and signs
Erythremia manifests in a number of characteristic signs of disease and pathologic complaints in affected patients. The leading symptoms of erythremia consist of elevated body temperature and enlarged liver and spleen. Since people affected by erythremia suffer from a deficiency of platelets, the tendency to bleeding increases, for example in minor injuries or inside the body. As a result, erythremia is usually accompanied by severe anemia. This also increases the risk of infectious diseases for those suffering from erythremia. Both the spleen and liver swell significantly in erythremia and are a typical feature of acute erythremia. The reduced percentage of granulocytes in the blood also increases the likelihood of contracting infections for patients with erythremia. In the absence of appropriate and timely therapy for erythremia, affected individuals often die after a few months as a result of the disease symptoms and associated complications. Far less common than the acute form is chronic erythremia. Unlike the acute type of erythremia, this disease is often much milder. Sometimes it is difficult to distinguish it from sideroblastic anemia, because in both diseases there is a disorder of iron utilization. In chronic erythremia, however, the erythroblasts are subject to lesser abnormalities.
Diagnosis
The first signs of erythremia prompt patients to consult a physician immediately. In the presence of erythremia, it should always be remembered that the disease is often fatal to those affected without adequate treatment. A general practitioner initially notes the complaint and usually refers the patient to a hematologist or comparable specialist. During the anamnesis, the treating specialist identifies the presenting symptoms and inquires about the onset of the complaints. The further procedure in the diagnosis of erythema is largely similar to those used in other forms of leukemia. Thus, the clinical examination of erythremia focuses primarily on a puncture of the bone marrow and various analyses of the blood. The diagnosis of erythremia is based predominantly on the results of bone marrow puncture. Here, the erythropoiesis shows a shift to the left.The differential diagnosis of erythema is often difficult, and the physician must distinguish the disease from other subtypes of leukemia.
Complications
In general, erythremia results in a markedly elevated body temperature. This is also associated with enlargement of the liver and spleen, so there may be pain in these areas. The affected person suffers from an increased risk of bleeding. Even minor injuries can lead to severe bleeding, which can also spread internally. The affected person also suffers from an increased risk of infectious diseases. If erythema is not treated properly or quickly enough, death usually results. The treatment itself is carried out with the help of drugs and supported by blood transfusions. The patient must be prepared for a long stay in a hospital. In rare cases, the blood transfusions cause complications. In order to slow down the growth of cancer, patients also receive cytostatic drugs. In most cases, erythema can be limited and completely treated with early treatment. However, the patient must undergo regular check-ups with a doctor. Treatment does not exclude the possibility that erythremia may recur later in life. With proper treatment, life expectancy is not reduced.
When should you see a doctor?
Unfortunately, in many cases, the symptoms of erythremia are not particularly characteristic and do not directly indicate the disease. However, early diagnosis increases the chances of a complete cure of the disease. A doctor should be consulted if the affected person suffers from an increased body temperature over a longer period of time. A strong tendency to bleed may also indicate erythremia. Furthermore, symptoms of the disease include an enlarged spleen and liver, which in some cases can lead to pain. Anemia also presents itself, which can lead to permanent fatigue and exhaustion of the patient. If these complaints occur without a particular reason and very frequently, an examination by a physician is always necessary. As a rule, erythraemia can be detected directly by a general practitioner. In children, a pediatrician can also be consulted. Further treatment then depends on the respective underlying disease, so that the help of other specialists is necessary in this case.
Treatment and therapy
Treatment of erythremia begins as soon as possible after the diagnosis is made. In most cases, therapeutic measures take place in a specialized center. Treatment of erythremia focuses on blood transfusions as well as 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 last only for a limited time, multiple transfusions at fixed intervals are usually required. Patients also receive cytostatic drugs. These are medical agents that slow down the growth of tumors. It is important that patients receive continuous care to monitor the success of the treatment measures and to improve the prognosis of erythropoiesis.
Outlook and prognosis
Without treatment, erythremia is not curable and always leads to death. Acute erythremia is fatal after several months if left untreated. In chronic erythremia, there is still an average life expectancy of about two years under these conditions. However, with treatment, good success can be achieved in prolonging life expectancy. Therapy consists mainly of the use of cytostatics and blood transfusions. Cytostatics inhibit the growth of cancer cells. Complete remission of cancer cells can be achieved in about 70 percent of patients. However, the older the patients are, the worse they respond to chemotherapy with cytostatics. Studies have shown that in patients over 60 years of age, the rate of complete remission is only between 30 and 60 percent. However, complete remission does not necessarily mean a complete cure. Blood tests merely show that no more cancer cells can be detected.However, these are not completely killed off in most patients, so there is a high relapse rate. Only 15 to 25 percent of all patients achieve a long-term remission, so that they can be considered cured. Whether these are true cures has not yet been clearly established. A complete cure can only be achieved by stem cell transplantation. However, this is only performed in exceptional cases in younger patients or in patients with poor response to chemotherapy. Stem cell transplantation carries the risk of severe side effects due to immune reactions.
Prevention
Specific ways to effectively prevent erythema have not been tested. Although certain mechanisms of the pathogenesis of erythremia have been studied, they are largely beyond the control of medicine.
Follow-up
In most cases of erythremia, the affected person has very limited options for follow-up care. The patient is primarily dependent on medical treatment by a physician to prevent further complications. Early diagnosis with early treatment is particularly important in order to treat the disease properly. Since the affected persons are dependent on transfusions due to the erythraemia, these should be carried out regularly. Furthermore, infections and other diseases should also be avoided as much as possible in order not to weaken and strain the immune system even more. Since erythraemia can also contribute to the development of tumors, regular examinations should be performed to detect and treat them at an early stage. The patient’s life expectancy may also be limited by the disease. Contact with other people affected by the disease can also be useful and have a positive effect on the further course of the disease. In the case of psychological upsets, intensive and loving care by parents is very important to alleviate these complaints.
This is what you can do yourself
If erythema is suspected, those affected must consult a physician immediately. Under no circumstances should you try to treat the disease yourself. Acute erythremia can lead to death within a few months. Therefore, the most important self-help measure is to recognize the symptoms and seek professional medical help promptly. Typical of the disease is an elevated body temperature associated with an enlarged liver and spleen. The organ enlargements are usually not noticeable to the affected persons themselves. However, due to a lack of platelets, the disease is also associated with severely impaired wound healing and pronounced anemia. Anyone who feels constantly tired and listless, is very pale, and also notices that even minor injuries bleed heavily and for a long time, should definitely consult a doctor immediately. Patients are usually treated with blood transfusions and the administration of cytostatic drugs. In naturopathy, natural cytostatics such as amygdalin from stone fruit seeds are often used. The effectiveness of such agents is questionable. Their use, however, after consultation with the attending physician, usually harmless. Other measures recommended in naturopathy for leukemia, such as a change in diet, can improve the general condition and support the therapy of the disease.
