Myeloproliferative disorders are malignant diseases of the hematopoietic system. The disease regulatory system is the monoclonal proliferation of one or more hematopoietic cell lines. The therapy depends on the particular illness in the individual case and can include blood transfusions, blood washing, medication and bone marrow transplants.

What are myeloproliferative disorders?

One of the main blood-forming organ is the medulla ossium, meaning the bone marrow. Together with the liver and spleen, it forms the human blood-forming system. Different diseases can affect the hematopoietic system. The collective term for malignant haematological diseases, for example, corresponds to a heterogeneous disease group with a malignant nature, which affects the blood-forming system.

Malignant haematological diseases include the subgroup of myeloproliferative disorders. This disease group is characterized by a monoclonal proliferation of stem cells in the bone marrow. In the literature, the corresponding diseases sometimes include myeloproliferative neoplasms.

The American hematologist Dameshek first proposed the concept of myeloproliferative syndromes for malignant diseases of the blood system, including diseases such as chronic myeloid leukemia. Meanwhile, the disease group of myeloproliferativen diseases has prevailed, which is based on a malignant degeneration of hematopoietic cells of the myeloid series. The group includes more than ten diseases, including polycythemia vera.

causes

On which causes a myeloproliferative disease is based, has not been conclusively clarified. According to speculation, risk factors such as ionizing radiation or chemical noxae cause the diseases of the hematopoietic system. The chemical noxae include scientists in this context, especially benzene and alkylating agents.

Although these noxae all have been shown to produce related phenomena, their association with the noxae is not immediately apparent in most cases of myeloproliferative disorders. Researchers have now at least agreed on the suspicion that previously unknown Noxen cause mutations in the genome.

These mutations should correspond to chromosome aberrations, ie anomalies in the genetically chromosomal genetic material. The anomalies are currently considered by researchers to be the primary cause of the disease. The hypothesis is supported by previously reported case reports of myeloproliferative disorders. For example, in many cases of polycythemia vera a mutation in the Janus kinase 2 gene JAK2 is present.

Symptoms, complaints and signs

The symptoms of myeloproliferative disorders can vary in severity and depend on the specific condition in each case. However, some complaints are common to most diseases in the group. In addition to leukocytosis, for example, erythrocytosis or thrombocytosis may occur.

This is an oversupply of certain blood cells. Especially in the early stages of myeloproliferative diseases, the three phenomena mentioned can occur simultaneously. In addition, patients often suffer from basophilia. An equally common symptom is splenomegaly. In many cases, fibrosis of the bone marrow also occurs, and this symptom is mainly characterized by osteomyelosclerosis.

Apart from fibrosis, this syndrome is also associated with extramedullary haematopoiesis. In extreme cases, a transition to a life-threatening blast thrust occurs in the course, especially in diseases such as CML. Depending on the particular illness, many other symptoms may occur in individual cases. The presence of all symptoms mentioned here is not mandatory for the diagnosis of myeloproliferative disease.

Diagnosis and disease course

The diagnosis of myeloproliferative disease is often difficult, especially in the early stages. A clear assignment of the symptoms is usually not possible in the early stages. In some cases, individual diseases from the disease group also overlap with each other, making the assignment even more difficult.

For example, polycythemia vera often occurs with or gets into osteomyelosclerosis. The disease course of the diseases is chronic and is subject to a certain progression. This means that the severity of the disease increases over time and therefore has a rather unfavorable prognosis.

When should you go to the doctor?

Disorders of the blood circulation or irregularities of the heart rhythm must be presented to a doctor. If there are restrictions on the possibilities of movement or swelling of the upper body, there is cause for concern. General dysfunctions, discomfort with digestion or inner restlessness are signs of a disease. A visit to a doctor is necessary as soon as the symptoms persist unabated or increase in intensity over a longer period of time. If the person complains of a feeling of illness, malaise or insomnia, he should consult a doctor. Sudden sweats or heavy night sweat production in spite of optimal sleeping conditions should be presented to a doctor.

An internal cold or heat development as well as an increased body temperature are indications of the organism for a present health irregularity. Headaches, disorders of concentration or a decrease in performance must be medically clarified. Changes in the muscular system, abnormal reaction of the body in contact with basal preparations and a decrease in body weight must be examined by a physician. If sports activities or everyday operations can no longer be performed, a doctor should be consulted. A persistent internal weakness, a general malaise or mental problems must be discussed with a doctor. Often, a serious illness hides behind the symptoms, where there is an immediate need for action. For a diagnosis to be made, a doctor's visit should take place.

Treatment and therapy

The treatment of myeloproliferative disease is symptomatic and depends on the disease in each case. Causal treatment is currently unavailable to patients. This means that the cause of the diseases can not be resolved. So far, science has not even agreed on the cause.

Until the disease is not clearly understood, no causal treatment options will be available. In diseases such as CML, the focus of symptomatic therapy is on conservative drug treatment approaches. The tyrosine kinase activity of the patients should be inhibited.

For this purpose, the affected person is administered, for example, the tyrosine kinase inhibitor imatinib. In addition, patients with chronic myeloid leukemia often receive drug therapy with hydroxycarbamide to normalize the white blood cell count. For the treatment of PV different measures are in use.

Bloodletting and apheresis reduce erythrocytes and other cellular blood components. In addition, thrombocyte aggregation inhibition occurs to prevent thrombosis. Oral antiplatelet agents such as acetylsalicylic acid are the drug of choice. Chemotherapy is indicated only if the high number of leukocytes or platelets causes thrombosis or embolism.

If hypereosinophilic syndrome is present, imatinib is the treatment of choice. To prevent embolism, oral anticoagulation is recommended. Patients with OMF are observed by hematologists and are usually treated by three approaches. In addition to bone marrow transplantation, drugs such as androgens, hydroxyurea, erythropoietin or ruxolitinib are available. The third component of the therapy is regular blood transfusions.

Outlook & Forecast

The myeloproliferative disorder has an unfavorable prognosis. It is a malignant disease that is difficult to treat. Without comprehensive medical care, further prospects deteriorate to a significant degree. The general life expectancy is reduced for the person concerned. The earlier a position of a diagnosis takes place, the sooner a start of therapy is possible. This increases the likelihood of a positive development in the further course of the disease.

Nevertheless, the challenge in treatment is coping with the generally progressive development of the disease. Since at the same time the cause of the disorder has not yet been completely clarified, physicians decide on the next treatment steps based on their individual situation. The illness represents a strong mental and emotional burden for the person concerned. In many cases, the overall circumstances lead to the development of a psychological sequelae.

The body of the patient is often so weakened that used therapeutic approaches do not bring the desired success. Although alleviation of many ailments is achieved, it is still difficult to predict how the person's personal development will develop. In addition to drug therapy, regular blood transfusions are necessary to improve general well-being. Overall, the susceptibility to infection and thus the risk of further health disorders increases. In some patients, bone marrow transplantation is the last chance to improve overall development.

prevention

So far, myeloproliferative diseases can not be prevented very promisingly, since the causes of the disease's development have not yet been conclusively clarified.

aftercare

Myeloproliferative disorders, such as polycythemia vera, require extensive follow-up care. Patients with this diagnosis must undergo periodic bloodletting. This reduces the number of blood cells and controls the blood levels. If the blood values ​​of the affected person are well adjusted, he has taken the first step.

Afterwards you have to think long term. The success of the therapy has to be preserved. In addition, follow-up includes making life with the condition as positive as possible. In both cases, patients should be in regular contact with their attending physician.

For myeloproliferative disorders, regular follow-up and follow-up are extremely important. Examination appointments are used to check the success of the therapy. On the basis of physical well-being, the doctor optimizes the therapy and adapts it to individual needs.

If patients feel uncomfortable between check-ups, they should contact their physician immediately. It is not advisable to wait until the next appointment. Patients do not have to accept any complaints. The doctor can already help with simple means.

In addition, he checks the respective complaints and accordingly initiates additional investigations. In addition, other aspects are to be considered, which go beyond the physical limitations. A visit to the psychologist may be beneficial if the illness also affects the patient's psyche.

You can do that yourself

Since myeloproliferative disease is a genetic disease, self-help options are limited. Treatment can be symptomatic. Nevertheless, apart from the medical treatment, individual measures to improve the quality of life can and should be found.

The focus here is on slowing down a negative course of the disease and maintaining the independence of the patients. In order to achieve this, various methods can be used, from which the most suitable approach for the diseased person is selected. Alternative therapies such as meditation, yoga or other physical exercises can help with pain relief and reduce stress caused by illness. Doctors, psychotherapists or occupational therapists can give instructions for exercises that are carried out independently at home. Important for the success of such methods is the continuous repetition. Only in this way can the preservation of performance become possible.

Since the course of the disease is very different, it can help to try different such measures. In general, it is advisable to keep the psychosocial environment of patients in the spotlight. An intact social network provides support and can help manage the effects of myeloproliferative disease.

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