Chronic myeloid leukemia (CML) is a specific subtype of leukemia in which the white blood cells in the blood become diseased and have a harmful effect on the entire body. But how exactly can CML be diagnosed? And how can chronic myeloid leukemia be treated?
What is chronic myeloid leukemia?
Chronic myeloid leukemia involves white blood cells called leukocytes. These blood cells are made from stem cells found in the bone marrow. The function of the white blood cells is, on the one hand, to destroy the pathogens and cell debris, and on the other hand, to provide immune defense. In the case of a disease with CML, the leukocytes cannot mature completely and are therefore functionless. At the same time, there is a strong and continuous proliferation of the defective leukocytes in the blood and bone marrow. If chronic myeloid leukemia progresses, these leukemic cells displace the healthy blood cells, causing an acute shortage of red and functional white platelets, as well as platelets. The disease usually progresses asymptomatically over several years and is therefore difficult to detect in the early stages, the chronic phase. CML should be distinguished from acute myeloid leukemia.
Causes
The cause of chronic myeloid leukemia disease is usually considered to be a genetic disorder of the genetic material. In CML, this is usually the so-called Philadelphia chromosome, which is shortened, causing excessive activity of tyrosine kinase. This enzyme is then responsible for the drastic increase in the number of leukocytes in the blood. Physicians also name benzene, ionizing and radioactive radiation, and viruses as risk factors for CML. However, the exact causes are still unknown despite the current state of medical research.
Symptoms, complaints, and signs
Chronic myeloid leukemia progresses insidiously and often causes no symptoms for years. Early in the disease, routine blood tests may reveal an increase in white blood cells (leukocytes) and the presence of immature blood cells. The first noticeable symptoms are often reduced performance, fatigue, depressive moods and loss of appetite – however, these complaints are very unspecific and can equally be caused by other, usually harmless health disorders. A clearer indication is a feeling of pressure in the upper abdomen caused by the palpably enlarged spleen. Occasionally, this also causes back pain. If the disease progresses, an increased tendency to bleed is often noticeable by bleeding gums, nosebleeds and bleeding into the skin. A deficiency of red blood cells is manifested by conspicuous pallor of the skin and a marked drop in performance; shortness of breath and an increased pulse rate are also possible. The weakening of the immune system results in an increased susceptibility to infections. Common symptoms also include fever without an identifiable cause, increased night sweats, and unwanted weight loss. Chronic myeloid leukemia should be considered if these symptoms recur or persist for a long time. Without treatment, symptoms usually worsen as the disease progresses, which can vary greatly from patient to patient.
Diagnosis and course
If chronic myeloid leukemia is suspected, the first step is to thoroughly examine the blood count in the laboratory. A bone marrow biopsy is also required to then combat the disease as quickly and effectively as possible. The definitive proof of CML, however, is the evidence of the presence of the Philadelphia chromosome. CML begins with the often symptomless chronic phase. This is followed by the so-called acceleration phase. This stage is primarily characterized by a deterioration of the blood count, which increases the bleeding tendency, while at the same time the immune system deteriorates. Affected individuals then tend to experience shortness of breath, pallor, heart palpitations and a drop in performance. This stage is usually followed by the so-called blast crisis, which manifests itself in the fact that the immature leukocytes, the blasts, are present in even greater numbers in the blood. Failure to treat CML at this stage can be fatal for the patient within a very short time.
Complications
Chronic myeloid leukemia results in unformed forms of leukocytes. It is therefore considered a neoplastic disease within the hematopoietic system. The symptom is not subject to hereditary predisposition and is not transmissible in the manner of infection. The changes directly affect the hematopoietic stem cells in the bone marrow. The pathogenesis is thought to be a genetic change caused by external circumstances. Possibly ionizing radiation, chemotherapeutic agents, benzene or viruses could have a triggering involvement. To date, all those affected by chronic myeloid leukemia have the Philadelphia chromosome. The symptom appears between the ages of forty and sixty, and the majority of sufferers are male. At the beginning, the symptoms are often misinterpreted. Which carries various risks of complications. In general, those affected complain of sweating, fever attacks, severe headaches and a considerable reduction in performance. The immune balance drops and the risk of infection increases. If the symptom is not medically clarified in time, leukemic thrombi can form. The spleen enlarges and pain develops in the upper abdomen. In some cases, a full-blown splenic infarction occurs. With the help of the informative blood count and the differential diagnosis, a normalization of the condition as well as a further spreading of the disease is aimed at. Depending on the case, stem cell transplantation, chemotherapy or preparations of the tyrosine kinase inhibitor group are used. If the patient is too debilitated, circulatory problems may occur as complication sequelae.
When should you see a doctor?
If chronic myeloid leukemia is suspected, a physician should be consulted in any case. In particular, pallor, fatigue, and unusual blood loss are among the symptoms that should definitely be clarified by a medical professional. A feeling of pressure in the left upper abdomen indicates advanced leukemia – in this case a doctor should be consulted immediately. Bleeding, increasing pain in the stomach area and the characteristic fever, which occurs independently of an infection, also require immediate clarification by the family doctor or a specialist. People who have already had cancer are particularly at risk. An unhealthy lifestyle and exposure to environmental toxins and pollutants also appear to be associated with the development of chronic myeloid leukemia. Those who belong to these risk groups should talk to their family doctor at the first warning signs. If there is a concrete suspicion, the oncologist or an internist can be consulted. If complications are already apparent, 112 or the medical emergency service should be contacted.
Treatment and therapy
Chronic myeloid leukemia is best fought with the help of tumor-specific drugs, so that in numerous cases the chances of cure are very high. These drugs, called tyrosine kinase inhibitors, inhibit the enzyme tyrosine kinase, causing leukemia cells to be pushed back. During treatment, patients may experience minor symptoms, such as fluid retention, muscle cramps or even nausea. It is important for patients to continue to have their blood and bone marrow checked regularly so that the success of the treatment can be seen at an early stage. In addition, this treatment method must be carried out permanently over a longer period of time in order to bring about a complete cure. If treatment with tyrosine kinase inhibitors is discontinued early, relapse often occurs. However, if the tyrosine kinase inhibitors do not work in the patient suffering from CML, bone marrow or stem cell transplantation is considered. However, this requires a suitable, healthy donor. Before the donor’s blood stem cells are injected, however, the patient must undergo high-dose chemotherapy to ensure that all leukemia cells are destroyed. However, transplantation must be thoroughly considered in advance because it can pose numerous risks.
Outlook and prognosis
Before the treatment options that exist today for chronic myeloid leukemia, the prognosis of the disease was very poor. Without treatment, the average life expectancy of affected patients is only three to four years. The disease typically then always proceeds according to the scheme of chronic phase, acceleration phase, and blast crisis.Once the blast crisis phase is reached, life expectancy is only a few weeks. With the drug therapy with tyrosine kinase inhibitors (TKIs) that is possible today, the chronic phase can be stabilized to such an extent that even a normal life expectancy is possible. By inhibiting tyrosine kinase activity, the ongoing proliferation of immature leukocytes is stopped. In some patients, after this treatment, the mutated multipotent hematopoietic progenitor cells can no longer be detected with the detection methods available today. However, it is not yet clear whether a complete cure is then achieved. Discontinuation of treatment could lead to a resurgence of the disease. In rare cases, however, drug therapy does not work. In such cases, stem cell transplantation must be performed, which leads to a complete cure, but can reduce life expectancy due to the side effects (rejection reactions, infections). Thus, lifelong therapy with tyrosine kinase inhibitors without complete cure has been shown to be more beneficial for the patient in many cases than stem cell therapy with complete cure.
Prevention
Unfortunately, true preventive measures cannot be taken for chronic myeloid leukemia. However, in the case of the disease, it is necessary that they administered drugs are taken continuously for a long period of time, so that a relapse can be avoided. The survival rate of CML, depending on the particular treatment method, is between 40 and 55 percent over ten years.
Follow-up
Chronic myeloid leukemia (AML) is a rare, malignant blood disorder. The uncontrolled proliferation of white blood cells is caused by impulses from the spinal cord. Because the blood and spinal cord are important vital centers, medical monitoring is essential after acute treatment of chronic myeloid leukemia. Young and older children also suffer from AML. A hematology specialist must regularly examine those with chronic myeloid leukemia. This is usually a specialist in internal medicine with additional training. As long as treatment continues, patients should see their hematologist every three months, and later every six months. If the symptoms worsen, the physician should be consulted immediately. Close follow-up is advisable because leukemia relapse can occur. Social exclusion or psychological problems should be taken into account during follow-up. The reason for the long aftercare is to be seen in the intensity of the therapies. The prescribed leukemia drugs are highly dosed. There may be radioactive irradiation. In addition, the knowledge that the disease cannot be cured weighs heavily on many people. In the acute phase, the focus is on therapy. Only after the end of acute treatment is the disease processed in its full dimension. Now the mental stresses of chronic myeloid leukemia are more in the foreground.
What you can do yourself
Chronic myeloid leukemia progresses slowly and can usually be influenced well by individually tailored drug therapy. Affected patients can therefore usually maintain a largely normal daily life and a high quality of life for a long time. The prerequisite for this is regular use of the medication prescribed by the doctor and consistent attendance at the follow-up appointments. However, the diagnosis can put a great strain on the patient’s own psyche and on his or her relationship with others, which is why coping with the disease is so important:
An open approach to the disease and thorough information about causes, treatment options and possible progression makes it easier to adjust to the changed situation. In addition to the attending physicians, psychosocial and psycho-oncological counseling centers can also provide support, and the exchange with other affected persons in a self-help group can also be helpful. A healthy lifestyle with a balanced diet and moderate exercise strengthens the immune system and can contribute greatly to physical and psychological well-being. In everyday life, sufferers should plan regular rest breaks and adapt the demands as far as possible to their own ability.Particularly at the beginning of therapy, susceptibility to infection may be increased: increased hygiene measures such as thorough hand washing, avoiding raw foods, and avoiding crowds will protect against infection.
