The myelodysplastic syndrome, MDS for short, describes various diseases of the blood or the hematopoietic system, which prevent the full development and functionality of healthy blood cells through genetic modification and in this way attack and weaken the organism. The probability of developing myelodysplastic syndrome increases with age and increases sharply from the age of 60.
What is myelodysplastic syndrome?
Contrary to popular misconception, myelodysplastic syndrome is not a blood cancer (leukemia). However, since acute myeloid leukemia (AML) can occur as a consequence in some cases, it has been given synonyms such as insidious leukemia or preleukemia. See eshaoxing for Camptodactyly Definition and Meaning.
A parallel to leukemia is that the bone marrow, as the center of blood formation, is also directly affected in the myelodysplastic syndrome and its essential function for the organism is impaired.
In this disease, the bone marrow is no longer able to produce enough white blood cells (leukocytes), red blood cells (erythrocytes) and platelets (thrombocytes) to ensure the transport of oxygen, maintenance of the immune system and proper blood clotting.
Causes
In nine out of ten cases, no direct reason for the development of a myelodysplastic syndrome can be identified. The rest is either the result of radiation or chemotherapy, such as that used in cancer patients, as so-called secondary myelodysplastic syndromes, or the effects of harmful and mostly toxic foreign substances such as benzene, which is contained in petrol, for example.
In addition, it is assumed that frequently consumed goods such as cigarette smoke, hair dyes, pesticides or alcohol can also contribute to the development of a myelodysplastic syndrome.
However, this theory has not yet been proven. However, a hereditary predisposition to myelodysplastic syndrome and transmission of the disease from person to person are completely excluded.
Symptoms, Ailments & Signs
About half of those affected have no symptoms at all and the disease is only discovered by chance. In patients with symptoms, the signs triggered by the anemia are particularly important. If the formation of red blood cells is disturbed, this leads to a lack of oxygen. Those affected feel tired and listless, and their ability to perform and concentrate decreases significantly.
If they have to exert themselves, they quickly run out of breath. Shortness of breath and sometimes rapid heartbeat (tachycardia) occur. Dizziness can also occur. The skin color is strikingly pale. However, the formation of white blood cells can also be impaired. This is reflected in a weakened immune system and the recurring infections caused by this, which can be accompanied by fever.
In some cases, the disease reduces the number of platelets in the blood. Since these are responsible for blood clotting, injuries bleed more and longer than normal. Bleeding gums are common. Petechiae can also form. These are small punctiform hemorrhages in the skin. Another symptom is enlargement of the spleen. Since the spleen has to work harder due to the lack of blood platelets, it increases in volume. The liver can also enlarge, which is noticeable in a feeling of pressure on the upper right side of the abdomen.
Diagnosis & History
The first signs of the occurrence of a myelodysplastic syndrome are very similar to those of anemia (anaemia), in which there are no longer enough red blood cells in the blood to distribute oxygen sufficiently quickly in the organism and this leads to symptoms such as shortness of breath, paleness, fatigue, dizziness, headaches, increased heart rate and ringing in the ears.
Due to the lack of white blood cells, infections can also occur more frequently, which can not be combated even by targeted treatment with antibiotics. As a result of the lack of sufficient blood platelets, difficult to stop and unusually heavy bleeding, for example in the case of small cuts or surgical interventions, can also often be observed. Frequent bruising or blood in the urine and stool can also be the first sign of myelodysplastic syndrome.
If there is a suspicion, detailed blood tests are usually carried out and the blood values are analyzed for deviations and abnormalities. In addition, a sample of bone marrow is taken from the hip and examined for changes in the chromosomes, which occur in about 60 percent of cases. Although myelodysplastic syndrome does not affect the body quite as quickly and devastatingly as leukemia, therapy should be started as soon as it is diagnosed, otherwise it can be caused by infections, such as those of the lungs or intestines, or by an infection acute leukemia developing from the myelodysplastic syndrome can become life-threatening.
Complications
This syndrome primarily leads to severe anemia. This generally has a very negative effect on the state of health of the patient and can also significantly reduce life expectancy. Due to the anemia, those affected appear very tired and exhausted and no longer actively participate in life.
There is also weakness and the patient’s resilience also decreases. Those affected can no longer concentrate and appear very pale. The syndrome also leads to heart palpitations and dizziness. Due to the reduced blood circulation, those affected also suffer from ringing in the ears and headaches.
The quality of life is significantly reduced and the patient often becomes irritable. Even small wounds or cuts can cause heavy bleeding, and blood can also appear in the urine. As a rule, a complete cure of the syndrome is possible only through the transplantation of stem cells.
Furthermore, those affected are dependent on regular transfusions in order not to die from the symptoms. In severe cases, chemotherapy is also necessary, but this can lead to various side effects. Without treatment of the syndrome, there is a significant reduction in the life expectancy of those affected.
When should you go to the doctor?
Fatigue, rapid fatigue and an increased need for sleep are indications of the body for an existing irregularity. If the symptoms persist for a long time or if they become more intense, a doctor’s visit is required. Disorders of concentration, attention or memory should be examined and clarified. If the performance level drops and the everyday requirements can no longer be met as a result, a doctor should be consulted. In case of a lack of the usual resilience, a pale skin or an inner weakness, a doctor’s visit is necessary.
If there are disturbances in the heart rhythm, heart palpitations, dizziness or unsteady gait, a doctor’s visit is recommended. A raised body temperature, a general malaise or a feeling of illness should be presented to a doctor. These are warning signals from the body that require action. Spontaneous bleeding of the gums, changes in the appearance of the skin and incomprehensible bruising are signs of a health impairment.
If the person affected notices swelling in the upper body or general functional disorders, they need medical help. Sensory disturbances in the upper body, hypersensitivity to touch or pressure indicate an irregularity in the organism. If you have these symptoms, you should consult a doctor as soon as possible so that a treatment plan to alleviate the symptoms can be drawn up. In addition, serious and acute illnesses must be excluded.
Treatment & Therapy
A myelodysplastic syndrome can ultimately only be completely cured by a successful stem cell transplant. All other treatment methods are only palliative, i.e. they alleviate the symptoms of the disease.
The lack of red blood cells can be combated, for example, with regular blood transfusions, and the lack of blood platelets with platelet concentrates. To prevent infections, vaccinations against influenza and pneumococci and preventive treatment with antibiotics are carried out. In addition, intensive personal hygiene and avoiding contact with possible disease carriers are recommended.
If the disease is already well advanced, chemotherapy is often used to eliminate the rapidly expanding cells in the bone marrow and blood, temporarily returning the blood count to a normal state. These methods vary greatly from patient to patient and must always be discussed and planned individually with the doctor treating you.
For this reason, the MDS Register Düsseldorf has existed since 2003, which has set itself the goal of being able to classify the course of the disease more individually and precisely and, on this basis, to be able to develop tailor-made therapies to combat the myelodysplastic syndrome.
Outlook & Forecast
The prognosis for myelodysplastic syndrome varies from patient to patient. It depends on the type and extent of the disease. During MDS, an increasing number of immature blood cells form. Therefore, there is a risk that the syndrome will turn into another form, the prognosis of which is even less favorable. This can be chronic myelomonocytic leukemia (CMML) or acute myeloid leukemia (AML).
Overall, the MDS prognosis is rather unfavorable. Factors such as complex chromosomal changes or a pronounced proportion of blast cells in the blood and a high decay value have a negative effect. The same applies to previous illnesses, poor general health or old age.
There are differences in the course and life expectancy, which depend on the respective risk group. The average life expectancy for high-risk MDS is five months. However, if stem cell therapy can be carried out, there is even a chance of a cure. This procedure is considered to be the only chance of recovery for MDS. If the risk of disease is lower, the patient has a life expectancy of up to 68 months. Up to 70 percent of all MDS sufferers die from bleeding, infections or the consequences of acute myeloid leukemia. In order to make the prognosis more favorable, it is important to strengthen the immune system. For this, the patient needs sufficient rest, a healthy diet and sporting activities.
Prevention
Due to great efforts in recent decades, the treatment of the myelodysplastic syndrome is becoming more and more effective and efficient, so that many of those affected today have a greatly increased chance of recovery or survival.
Aftercare
In most cases, those affected by this syndrome have only very few or limited direct follow-up measures available. First and foremost, a doctor should be contacted early on so that no further complications or symptoms can occur. Self-healing cannot occur.
An early diagnosis always has a very positive effect on the further course of the disease, so that the person affected should ideally see a doctor as soon as the first symptoms and signs of the disease appear. Those affected should protect themselves particularly well against various infections and inflammations in order to avoid complications.
The support and care of one’s own family and relatives is very important and has a positive effect on the further course of this disease. Psychological support can be helpful here to prevent depression and other mental upsets. In many cases, however, this disease reduces the life expectancy of those affected.
You can do that yourself
For those affected, it is primarily important to clarify what type of disease they are suffering from and what treatment options are available.
If the treatment is carried out by means of chemotherapy, this represents an enormous burden for the body. During this time, the organism has an increased need for nutrients, which can be partially covered by a change in diet. If this is not sufficient, a supportive therapy with micronutrients, which is tailored to the individual needs, must be carried out in cooperation with the doctor.
If the possibility of a stem cell transplant is in the air, the person concerned can set up donation events together with friends, family and colleagues, at which the population is specifically called upon to register in the bone marrow donor database. Even if there is no suitable donor among the newly registered ones, it can mean the longed-for donation for other affected people.
Regular blood transfusions are a common therapy to keep the side effects of the disease as low as possible. However, this inevitably leads to an excess of iron in the organism. In order to avoid damage to organs and tissues, this must be led out of the body with medication. The tablets required for this must be taken very conscientiously, even if side effects occur, since excess iron only causes symptoms when permanent damage to the organism has already occurred.
