Acute lymphoblastic leukemia is a pathological malignant neoplasm in the hematopoietic system. The disease originates in the bone marrow, where leukocytes are formed in large quantities (immature white blood cells). Acute lymphoblastic leukemia absolutely controls the processes of growth, uniform renewal, and the complex maturation of all human cells is lost. Leukocytes do not mature and do not become full-fledged and functional cells, however, their rapid and uncontrolled division occurs. Thus, the process of repression of healthy malignant cells in order to occupy their position in the bone marrow is marked. Therefore, he loses the ability to form healthy platelets, red blood cells and white blood cells. This causes anemia, infectious complications and frequent bleeding. Sometimes it is these symptoms that become the first signs of acute lymphoblastic leukemia.
All leukemias through the blood and lymphatic system spread throughout the body and affect almost all systems. Therefore, such an anomaly is considered to be a systemic malignant pathology and without timely, appropriate treatment, a lethal outcome may occur within a few months from the onset of the disease.
Acute lymphoblastic leukemia causes
In the development of this disease in humans, disorders in the compound and structural set of chromosomes are presumed, which can be caused by hereditary or acquired factors as a result of peculiar mutations in the genes. The main such factors include ionizing radiation, and chemical mutagens play an important role in the formation of acute lymphoblastic leukemia.
There is evidence that this pathological neoplasm is increasing among people who have been exposed to benzene or who have taken cytotoxic drugs such as Mustargen, Leikaran, Imuran, Sarcolysin, and Cyclophosphamide.
There are also suggestions that a hereditary factor predisposes the development of this pathology in the lymphatic tissue. In addition, there are descriptions of observations that relate to the dominant and recessive ways of inheriting acute lymphoblastic leukemia with a low incidence in certain ethnic groups and high in others. Basically, in such cases, the inheritance occurs not of the disease itself, but of the ability of chromosomes to undergo variability,
Acute lymphoblastic leukemia symptoms
For this cancer, there are several stages of its occurrence. These include: the pre-leukemia period, followed by the acute form, then remission occurs, after which the disease recurs and the last stage is terminal. Of course, the last three stages of acute lymphoblastic leukemia in most cases depend on the effectiveness of the treatment. And if the disease is cured, then the recurrent processes and the terminal stage are completely absent. And with primary resistance, alternations of remission with relapse are observed.
During the pre-leukemic period, the clinical signs of acute lymphoblastic leukemia are almost impossible to diagnose. But the acute period begins fairly quickly and is characterized by a variety of symptoms, which are characterized by processes of a strong increase in the pathological tissue and the suppression of the normal function of blood formation. This is manifested by blast transformation of the bone marrow, an increase in somatic organs and lymph nodes.
In adults, various forms of acute lymphoblastic leukemia are characterized by early intoxication symptoms, anorexia without significant weight loss, pain in the joints and bones. Further, there is an increase in organs such as the liver, spleen and lymph nodes, especially in the neck, in the armpit and in the groin. With enlarged submandibular lymph nodes, parotid and peri-orbital symptomatic complex Mikulich. In the course of the disease in many patients, pallor of the mucous membranes and skin is noted.
Also, acute lymphoblastic leukemia can manifest itself as a hemorrhagic syndrome, which is expressed by hemorrhages of a polymorphic nature from small to large, as well as by nosebleeds, renal and from the gastrointestinal tract. All these signs indicate the development of thrombocytopenia and anemia due to the suppression of normal blood formation.
Acute lymphoblastic leukemia is characterized by rare symptoms, such as leukemias, which form on the surface of the skin and in its fatty tissue. Necrotic changes of the skin, mucous membranes of the mouth and intestines may also be present.
During the progression of the pathological process, acute lymphoblastic leukemia spreads to many organs. These include the central nervous system, and lungs, and organs of vision, as well as the organs of the sexual sphere. Such clinical symptoms are more characteristic of recurrent malignant disease. But when acute lymphoblastic leukemia is characterized by the appearance of these signs at the beginning of the disease, this indicates a poor prognosis of pathology.
Acute lymphoblastic leukemia in children
This malignant neoplasm is considered the most common form of leukemia among adolescents and children, occurring in almost 80% of all cases. According to statistics, this pathology annually affects nearly 500 adolescents and children from birth to fourteen years.
Acute lymphoblastic leukemia can affect both children of any age and adults. Although much more often this disease is diagnosed among boys from one to five years than girls.
In children, acute lymphoblastic leukemia is caused by malignant lymphocyte mutations, as a result of which new cells stop their development. Such a process of arrest can occur at any stage of cell formation, therefore, acute lymphoblastic leukemia can manifest itself in various forms. All of them have different clinical course of the disease, and the tactics of treatment will also depend on these forms.
At the moment there are no exact reasons that would contribute to or influence the development of acute lymphoblastic leukemia in children. It is only known that the disease begins to develop when the precursors of lymphocytes are malignant. Such mutations change the entire hereditary material of the cell. But it remains largely incomprehensible why in some children these changes in the genes lead to the formation of pathology, while in other children they do not. This can also be explained by the fact that acute lymphoblastic leukemia is influenced not only by genetic predisposition, but also by external factors. Thus, this disease is considered multifactorial.
It is also known that diseases such as Fanconi anemia and Down syndrome increase the risk of developing this malignant pathology in children. In addition, X-ray radiation, radiation, chemical carcinogens, medications and certain viruses can contribute to the development of acute lymphoblastic leukemia in children.
Within a few weeks, the first clinical picture of the disease develops. Since the tumor pathological cells gradually affect the entire bone marrow and other organs, the symptoms of acute lymphoblastic leukemia in children are first manifested as lethargy, lack of interest in games, and pallor. This is due to the insufficient amount of red blood cells in the body of the child, which deliver oxygen to all cells. A lack of full-fledged lymphocytes and granulocytes weakens the immune system, which is a consequence of the development of frequent infections with feverish temperatures.
With no platelets, skin hemorrhages and mucosal bleeding occur. Malignant cells, moving throughout the body, cause not only changes in the blood, but also pain in various organs. The first appear pain in the bones of the hands and feet, after filling the bone marrow and bone cavities with leukemic cells. Sometimes these pains become so powerful that children cannot even walk. With the accumulation of abnormal cells in the lymph nodes, liver and spleen, pain in the abdomen. And when the disease spreads to the membranes of the brain, children complain of headaches, they have facial nerve paralysis, their vision is impaired and vomiting can occur.
Symptoms of acute lymphoblastic leukemia in different children can occur and manifest individually. Therefore, to clarify the diagnosis, it is necessary to undergo a full diagnosis of the disease.
If you suspect this anomaly, the pediatrician prescribes laboratory tests in the form of a detailed blood test. And already changes in the hemogram are forced to make a bone marrow puncture in the hospital for final confirmation of the diagnosis. To get more accurate information about the affected organs, ultrasound, x-rays, MRI, CT and scintigraphy of the skeleton, lumbar puncture are prescribed.
Acute lymphoblastic leukemia treatment
With a confirmed diagnosis, patients in a specialized clinic begin to apply the most modern and effective treatment. In the treatment of acute lymphoblastic leukemia, polychemotherapy is of paramount importance. To do this, patients are prescribed cytotoxic drugs that slow down and reduce the growth of cancer cells. Some patients additionally conduct radiation exposure to the central nervous system. And the use of high-dose chemotherapy makes it possible to perform stem cell transplantation. The main objective in such treatment of acute lymphoblastic leukemia is the possibility of the complete destruction of malignant leukemic cells throughout the body in order to restore full-fledged bone marrow work.
The duration and intensity of polychemotherapy, the need for radiation exposure of the nervous system, transplantation, as well as the outcome of treatment and prognosis will largely depend on the type of acute lymphoblastic leukemia, its spread throughout the patient’s body and how the disease is treatable.
Simultaneously with chemotherapy, accompanying therapy of acute lymphoblastic leukemia is prescribed, which is aimed at combating various infections, while eliminating the side effects after chemotherapy and symptoms of intoxication. For severe thrombocytopenia and anemia, blood substitutes are prescribed.
Bone marrow cell transplantation is one of the methods of treating a disease such as acute lymphoblastic leukemia. It is used for those patients who have complications in the presence of a chromosome of the Philadelphia type. An important point in the process of therapy is to prevent the accession of infection, due to the weakening of the immune system in patients. Acute lymphoblastic leukemia is less amenable to treatment in adults than in pediatric pathology.
Acute lymphoblastic leukemia prognosis
Today, about 70% of children who suffer from acute lymphoblastic leukemia, have a five-year remission, but in adults the duration of the remission period can be achieved only in 20%. For example, in Germany, out of 600 patients who first fell ill with this pathology, 90 have recurrent disease processes,
As a rule, there are very few chances for recovery after the return of acute lymphoblastic leukemia, although in some patients repeated therapy gives good results. Five-year survival with repeated manifestations of the disease is about 35%.
Statistical indicators cannot predict prognosis of malignant pathology. Acute lymphoblastic leukemia can both proceed and end completely unpredictably, even with the most favorable course or vice versa.