Myeloproliferative neoplasia is a group of different chronic diseases of the bone marrow and blood. Myeloproliferative neoplasia is malignant and relatively rare. In myeloproliferative neoplasia, the marrow of the bone produces an excess of blood cells. A cure for myeloproliferative neoplasia currently exists exclusively in the transplantation of stem cells.

What is myeloproliferative neoplasia?

Myeloproliferative neoplasia is also referred to as chronic myeloproliferative disease and is often referred to as CMPE by physicians. Myeloproliferative neoplasia is primarily affected by those cells of the bone marrow that play an essential role in the formation of new blood cells.

While various forms of myeloproliferative neoplasia exist, all manifestations of the disease share the excessive production of various blood components. These include, for example, the red and white blood cells as well as the platelets. The disturbed hematopoiesis either refers to a single type of blood substance or concerns several.

Risky for the patient with myeloproliferative neoplasia, especially the symptoms that result from the excess of certain blood cells. Possible complications include, for example, occlusions of vessels, blood clots, and an increased tendency to bleeding.

Particularly common types of myeloproliferative neoplasia are polycythemia vera, essential thrombocythemia, chronic myeloid leukemia, and primary myelofibrosis. In the majority of cases, there are certain genes present in the affected individuals that promote the development of myeloproliferative neoplasia.

causes

In principle, myeloproliferative neoplasia is a cancer. The causes are usually found in changes in the genes that lead to the malignant abnormalities in blood formation. These genetic mutations affect the cells of the bone marrow that produce the blood cells.

These changes occur either by accident or due to certain external factors, such as lifestyle risk factors, taking special medicines, or similar causes. Many people suffering from myeloproliferative neoplasia have gene mutations in the so-called Janus kinase 2.

These are proteins for controlling the division of cells. As a result of the disorder, the affected cells are no longer able to stop cell division. As a result, they duplicate themselves further and further. In addition, a number of other genetic mutations are responsible for the development of myeloproliferative neoplasia.

In general, myeloproliferative neoplasia is rare. The frequency of myeloproliferative neoplasia is estimated to be one to two cases per 100, 000 people. While myeloproliferative neoplasia occurs principally in persons of all age groups, the disease develops particularly often in advanced age. Male patients suffer from myeloproliferative neoplasia more often than women.

Symptoms, complaints & signs

The symptoms of myeloproliferative neoplasia occur slowly over a long period of time and are therefore often barely noticed by patients at first. Frequently, myeloproliferative neoplasia is therefore discovered by chance during routine blood analyzes. All symptoms of myeloproliferative neoplasia are caused by the excess of blood cells and platelets.

Thus, the persons are often knocked off and tired, suffer from headaches, dizziness and a disturbed circulation of the feet and hands. Sometimes myeloproliferative neoplasia affects the visual sense. For example, the increased tendency to bleed manifests itself in unusual bruises, petechiae and prolonged bleeding on minor injuries.

In female patients, the duration of the menstrual bleeding may be prolonged. In an advanced stage of myeloproliferative neoplasia, a feeling of pressure develops on the left side of the abdomen as the spleen enlarges. Many people also suffer from loss of appetite and weight loss. Even tinnitus and tinnitus as well as nocturnal sweat attacks and cramps in the calves are possible.

Diagnosis & disease course

Frequently, myeloproliferative neoplasia is randomly diagnosed when the blood is analyzed by laboratory tests during follow-up examinations. Significantly increased blood cell levels indicate myeloproliferative neoplasia. Sometimes certain proportions of blood substances are also reduced.

A subsequent examination of the abdomen by means of ultrasound shows the enlargement of the spleen. The patient is referred to a hematologist, who concretizes the diagnosis of myeloproliferative neoplasia and determines the exact type of the disease. Genetic analyzes allow the search for the so-called JAK2 gene, which largely secures the diagnosis of myeloproliferative neoplasia. In addition, the doctor examines the patient's bone marrow using the puncture procedure.

complications

In most cases, this disease is recognized and diagnosed late. For this reason, early treatment is usually not possible. The affected suffer from a permanent fatigue and also a fatigue. Likewise, the tiredness can not be compensated by sleep.

Likewise, it often comes to a strong dizziness and continue to headache. The quality of life of the patient is significantly limited by this disease. The extremities of those affected are no longer properly supplied with blood, so that they usually look cold. Also, the sense of sight is negatively affected by the disease, so that the patients can suffer from foggy vision or double vision.

Already with small injuries it comes to heavy bleeding, which in most cases does not stop by itself again. Furthermore, the disease also leads to a severe loss of weight and loss of appetite. During the night sufferers often suffer from sweats or panic attacks.

Ear noises can also occur and continue to negatively affect the patient's quality of life. The treatment of the disease is carried out with the help of medication. There are no further complications. However, a complete treatment of this disease is unfortunately not possible.

When should you go to the doctor?

The myeloproliferative neoplasia can cause very different symptoms, which, however, must always be clarified by a doctor. Already first warning signs such as tiredness, exhaustion or circulatory disturbances on hands and feet must be medically examined and treated, so that it does not come to further complications later. The same applies to dizziness, headaches and blurred vision and an increased tendency to bleed, which is characterized by small, punctate hemorrhages. If a feeling of pressure in the left upper abdomen is added, the disease may already be well advanced. At the latest then a doctor must be consulted, who can diagnose or exclude the suffering.

People who are chronically ill or have a disease of the immune system are particularly susceptible to the development of myeloproliferative neoplasia. Even people with conquered bone marrow diseases are among the risk groups and should inform the responsible doctor quickly. The treatment is provided by internists and specialists for diseases of the bone marrow. If weight loss occurs as a result of loss of appetite, a nutritionist must be consulted.

Treatment & Therapy

Basically, the myeloproliferative neoplasia is not curable so far, instead, the disease is treated based on their symptoms. For example, patients receive platelet aggregation inhibitors that counteract blood clots. This reduces the risk of vascular occlusion and thrombosis. Often the drug acetylsalicylic acid is used.

Aderlass is also particularly effective for the treatment of myeloproliferative neoplasia. By removing a certain amount of blood from the body, the symptoms usually improve at short notice. It is taken up to half a liter of blood per bloodletting.

In the long term, the lack of red blood cells causes iron deficiency, resulting in fewer blood cells. In addition to other drug therapies, there is the possibility of stem cell transplantation.

Outlook & Forecast

The prognosis is bad according to the current scientific state. Myeloproliferative neoplasia is considered incurable. Doctors can only alleviate the symptoms. It remains to be seen to what extent research will break new therapeutic pathways in the future. The problem is that the symptoms increase over time. Many patients suffer losses in their quality of life. Depending on its severity, myeloproliferative neoplasia can also shorten the lifespan.

Myeloproliferative neoplasia affects mainly adults around the age of 60 years. Only rarely do younger people get sick. Statistically, one to two new patients per 100, 000 inhabitants are added each year. Early diagnosis of myeloproliferative neoplasia is difficult because the first signs are nonspecific and may be related to other forms of disease. This fact worsens the initial situation and justifies the unfavorable forecast.

Doctors assess the prospects for the elderly as being particularly critical in the presence of thrombosis or leukocytosis. Standardized therapy concepts do not exist so far. Patients have to experiment in part, until arranged means show their effect. Risks and side effects can not be ruled out during treatment.

prevention

At present, myeloproliferative neoplasia can not be specifically prevented because most of the developmental factors of malignant disease are largely beyond the control of the patient.

You can do that yourself

Everyday management after diagnosis is mainly characterized by self-observation. In order to maintain the highest possible quality of life, it is necessary for the patients to regain a good body feeling and thus to increase their well-being.

Disease management also plays a central role in the lives of patients. Actively dealing with myeloproliferative neoplasia, informing oneself and not giving up has in most cases a positive effect on the quality of life. Sharing with other stakeholders through forums or in support groups can be invaluable. An exchange between the patients leads to more understanding and the feeling of not being alone. In addition to self-help groups, it makes sense to keep a diary or log. Write down those affected, what they experience, how they feel and what goes through their minds, they can later better classify these thoughts and sensitivities.

In addition, a protocol helps with self-observation. Changes after certain activities, discomfort after certain meals, or regaining abilities facilitate the regaining of the body sensation and also contribute to a more positive attitude. It is important for the patients to follow the small successes. It may also be helpful to discuss this with the doctor.

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