Acute myeloid leukemia (AML)
Acute myeloid leukemia (AML) is a malignant disease that starts from immature precursors of red blood cells, platelets and some white blood cells. AML is the most common form of acute leukemia in adults. Thanks to the intensive research work of the last decades, the treatment options and chances of recovery have improved significantly. The diagnosis of AML should therefore not lead to hopelessness. An early incurable disease has become curable in many cases!
Acute myeloid leukemia (AML) is a malignant disease of the blood-forming system (blood cancer) in which an early precursor of a myeloid cell degenerates and proliferates in an uncontrolled manner. Myeloid cells include red blood cells, platelets and some white blood cells. In healthy people, the proliferation and renewal of blood cells is strictly regulated. The AML has gotten this process out of control:
As a result of the changes in genetic material, the affected cell begins to divide and multiply unrestrained without, however, developing into normal, functioning blood cells. The resulting cells are called myeloid blasts. They spread rapidly in the bone marrow and hinder the formation of healthy blood cells there. The blasts can eventually be distributed in the body via the blood and infect and damage other organs.
Causes and frequency
Acute myeloid leukemia is not a hereditary disease and, like other forms of cancer, is neither contagious nor can it be transmitted to other people. The causes of AML are malignant, genetic changes in the bone marrow, which are acquired in the course of life. Risk factors include ionizing radiation and certain chemical substances. Even some medicines, which are used for the treatment of cancer itself, can in rare cases trigger years later a so-called secondary AML. Patients with various diseases of the blood or bone marrow (e.g., a myelodysplastic syndrome) or genetic diseases such as Down syndrome also have an increased risk of developing AML. In most cases, however, it remains completely unclear what has been the cause of the disease.
Acute myeloid leukemia is a rare disease with 3.5 new diagnoses annually per 100,000 inhabitants, but the most common form of acute leukemia in Germany. Men are more affected than women. In contrast to ALL, which occurs predominantly in children, AML is a disease of older people - about half of the patients are over 70 years old.
symptoms
The symptoms of AML usually develop within a few weeks. They are caused by the lack of normal blood cells on the one hand and by the involvement of organs with myeloid blasts on the other hand.
very common symptoms
Anemia (anemia, red blood cell depletion) causes paleness, fatigue, fatigue, decreased performance, shortness of breath, generalized weakness and malaise
Fever and / or increased susceptibility to infection due to reduction of white blood cells (leukopenia)
Abdominal pain and loss of appetite due to enlargement (organ involvement) of spleen and / or liver
Propagation of leukocytes (leukocytosis) by overproduction of lymphatic blasts
common symptoms
Reduction of thrombocytes (thrombocytopenia) causes bleeding (small punctate skin bleeding (petechiae), bruising, nosebleeds, prolonged bleeding, for example after a visit to the dentist or after injuries, prolonged menstrual bleeding in women, rarely even cerebral haemorrhage)
Swollen lymph nodes on the neck, in the armpits or in the groin
Joint and bone pain from the spread of blasts in the bones
rare symptomsInfestation of the brain, spinal cord or meninges with neurological changes such as headache, blurred vision, vomiting or nerve paralysis
Skin changes and chloromas (accumulation of blasts, for example, in the skin or bone marrow)
difficulty in breathing
Some patients have little discomfort and the leukemia is discovered by chance during a routine blood test.
All the symptoms described also occur in the context of other comparatively harmless diseases and in most cases have nothing to do with leukemia. For persistent symptoms, it is advisable to have the cause clarified by a doctor. If acute myeloid leukemia is present, therapy must begin as soon as possible.
Diagnosis of AML
If leukemia is suspected, extensive examinations of the blood and bone marrow are necessary to first confirm the diagnosis and then determine the type of leukemia. With the help of so-called imaging techniques, the extent of the disease is also determined.
If the family doctor / internist finds evidence of acute leukemia on the basis of the medical history and physical examinations, he will first examine the blood. Confirms the suspicion of leukemia, because e.g. If immature cells are found in the blood, if there is anemia or thrombocytopenia, the diagnosis must be additionally confirmed by analysis of the bone marrow. Such an examination is carried out at a specialized hospital by a specialist in blood and cancer (hematologist / oncologist). He removes bone marrow from the hip bone or sternum (bone marrow puncture) with a syringe under local anesthesia. This short, outpatient procedure can be a bit uncomfortable for the patient since it takes a few minutes for the bone marrow to get into the syringe.
Subsequent laboratory studies of the bone marrow include the biological characteristics, external cell characteristics, and genetic alterations of the affected cells. With the help of the analysis, in addition to the type of leukemia, the exact subtype of the disease can be determined. Since the subtypes of acute myeloid leukemia differ significantly in their disease progression, prognosis and response to different therapy, in-depth examination of the bone marrow is crucial to develop a suitable treatment plan.
If AML is detected, additional examinations may be required to exclude the involvement of other organs in the body with blasts, depending on the individual case. These include chest x-rays, computed tomography and ultrasound of the abdomen and heart. In exceptional cases, a sample is taken from the cerebrospinal fluid (lumbar puncture) to determine if there is leukemia in the brain.
In the further course after the start of therapy, bone marrow punctures and other check-ups are necessary. Thus, e.g. With the aid of MRD diagnostics, it can be checked whether the leukemia has completely resolved (remission).
Classification of AML
Acute myeloid leukemia is not a single disorder but rather a group of distinct subtypes that can be distinguished by their biological characteristics, external cell characteristics, and genetic alterations. The assignment to a specific subform plays an important role in the treatment decision, since the various subforms differ in terms of the course of the disease and the chances of recovery (prognosis) and are different in their sensitivity to chemotherapy. Basically, a distinction is made between primary and secondary AML: If the AML occurs independently and without previous bone marrow or cancer, one speaks of a primary or de novo AML. Secondary AML develops from another bone marrow disease (e.g., a myelodysplastic syndrome) or arises as a result of previous chemotherapy or radiotherapy. Since secondary AML is usually associated with multiple genetic changes, it has a worse prognosis than primary AML.
The classification of AML is nowadays mostly based on the WHO classification. This combines the older FAB classification, in which the blasts are subdivided according to their outer, microscopically visible features into eight subgroups (M0-M7), with genetic peculiarities of the leukemic cells. The external features as well as the underlying genetic changes can be determined by means of comparative laboratory investigations such as microscopy, cytochemistry, immunophenotyping, molecular genetics, cytogenetics or in situ hybridization.Acute promyelocytic leukemia (APL)
Acute promyelocytic leukemia (APL) is a rare subset of AML that is diagnosed in approximately 5% of AML patients. In this disease, a particular genetic alteration known as translocation t (15; 17) can be detected. It leads to the formation of the defective protein PML-RARA, which is causally involved in the development of the disease. The APL plays a special role in terms of disease progression, prognosis and treatment. The disease is particularly prevalent in younger patients and is associated with a good prognosis: Combining chemotherapy with all-trans-retinoic acid (ATRA), which stimulates leukemia cells to mature, today more than 75% of patients can be cured. The prerequisite, however, is the treatment in a specialized leukemia center, because the APL often causes a disruption of blood clotting and is therefore associated with a high rate of bleeding complications.
Treatment of acute myeloid leukemia
AML is a serious illness that, if left untreated, will kill within a few weeks. Therefore, it is extremely important that treatment is started immediately after diagnosis. The most important part of the treatment is chemotherapy with adjunctive therapy to treat the side effects. For this purpose, a bone marrow transplant can come in individual cases. Radiotherapy plays a minor role in AML. All components of the therapy serve to kill the leukemia cells as completely as possible throughout the body, so that the bone marrow can resume its original function - the formation of blood.
Treatment for AML should be performed at a clinic that has experience in the treatment of leukemias - especially AML. The clinic should have access to the latest research and be able to conduct necessary special examinations. Find out more about choosing the right clinic here. Since the treatment is tedious and stressful, patients and their relatives should be fully informed about the planned process. The attending physician will talk in detail with the patient and inform him about all treatment options.
chemotherapy
In chemotherapy, a patient receives drugs that are called cytostatic drugs and specifically inhibit the growth of leukemia cells. Since a single drug is usually not sufficient to destroy all blasts, combining several drugs with different modes of action. They are given as an infusion, syringe or in the form of tablets. The chemotherapy is divided into several cycles, separated by breaks (intervals), in which healthy cells can regenerate. To assess the success of the therapy, the bone marrow and other affected regions are checked at regular intervals.
stem cell
Another possibility of treatment is stem cell transplantation. The aim of this therapy is to replace the diseased bone marrow by healthy. In stem cell transplantation, purified blood stem cells from an appropriate donor (allogeneic) or, more rarely, the patient himself (autologous) are infused into the patient. For successful therapy, all leukemia cells must be killed beforehand. This is achieved by strong chemotherapy and radiation, which destroys not only the blasts but also the healthy cells in the bone marrow. Since this is a stressful and risky therapy, the patient must be eligible for a stem cell transplantation in terms of general condition and age. A stem cell transplantation is usually only performed if, due to the characteristics of the leukemia cells, it must be assumed that there is a high risk of relapse (recurrence) or if chemotherapy has not produced sufficient treatment success.treatment studies
The treatment of AML patients should be done as far as possible in the context of therapy studies. Here the patient gets access to the latest scientific findings and is treated with innovative medicines and according to current treatment strategies. Participation in a study does not mean that the application of the drugs used is experimental. Rather, the goal is to improve the treatment strategies of AML in the future. The decision as to which study is suitable for a patient must be discussed in detail with the attending physician. There are several criteria, e.g. Disease features, disease phase, pretreatment, age and risk factors play a role. The final decision to participate in the study is always made by the patient himself. In Germany, numerous clinics participate in the studies of the German study groups for adult AML. The current studies can be found in the German Leukemia Study Register.
Treatment options for a relapse
If AML relapses, chemotherapy will be used again first. In case of late relapses, the induction therapy can be repeated to once again achieve complete destruction of the leukemia cells (remission). In early relapses or when the disease is not responding to therapy (treatment failure), various options are available, e.g. Researched with new drugs in the context of therapy studies. Here it is important that the treating physicians are informed about all currently available treatment options. The goal is to achieve a complete response. Often, when age and general condition permit, and when a donor can be found, stem cell transplantation is performed.
aftercare
The number of healings has steadily increased in AML over the past decades. Regular follow-up is required. They include a general physical examination as well as blood and bone marrow checks. The aim is to detect signs of a relapse or late effects of the therapy at an early stage. With the distance from the diagnosis and the end of the therapy, the intervals between examinations are extended. The attending physician also advises on the possibility of a rehabilitation measure (rehab). It is usually funded by the health insurance companies and provides a good transitional opportunity to get back into a "normal" life.
Information brochure of the Kompetenznetz Leukämien
The Patient Brochure on Acute Myeloid Leukemia (AML) in adults in the leukemia competence network was developed in cooperation with the UCT Frankfurt and the Leukemia Aid Rhein-Main g.e.V. reissued. It takes into account the current state of research and explains the treatment options for adult AML. In particular, it contains refurbished information and facts about adult AML for patients and relatives. Important contents include: symptoms, diagnosis, treatment options and FAQs about AML.
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