Sharp limfoblastny leukosis
Sharp limfoblastny leukosis – the malignant defeat of system of blood formation which is followed by uncontrollable increase in quantity of limfoblast. It is shown by anemia, intoxication symptoms, increase in lymph nodes, the liver and spleen raised by bleeding and respiratory frustration. Because of decrease in immunity at a sharp limfoblastny leukosis infectious diseases often develop. Defeat of TsNS is possible. The diagnosis is exposed on the basis of clinical symptoms and these laboratory researches. Treatment – chemotherapy, radiotheraphy, bone marrow transplantation.
Sharp limfoblastny leukosis
The Sharp Limfoblastny Leukosis (SLL) – the most widespread oncological disease of children's age. The share of OLL makes 75-80% of total of cases of diseases of system of blood formation at children. The peak of incidence is the share of age of 1-6 years. Boys suffer more often than girls. Adult patients are ill 8-10 times less often than children. At patients of children's age the sharp limfoblastny leukosis arises initially, at adults quite often is a complication of a chronic lymphocytic leukosis. In the clinical manifestations OLL is similar to other sharp leukoses. Distinctive feature is more frequent defeat of covers of a head and spinal cord (neuroleukosis), in the absence of prevention developing at 30-50% of patients. Treatment is performed by experts in the field of oncology and hematology.
According to the WHO classification distinguish four OLL types: - - In - cellular, - In - cellular, V-cellular and T-cellular. V-cellular sharp limfoblastny leukoses make 80-85% of total of cases. The first peak of incidence is the share of age 3 years. In the subsequent the probability of development of OLL increases after 60 years. The T-cellular leukosis makes 15-20% of total of cases of a disease. The peak of incidence is the share of age of 15 years.
Reasons of a sharp limfoblastny leukosis
Formation of a malignant clone – group of the cages having ability to uncontrollable reproduction is an immediate cause of a sharp limfoblastny leukosis. The clone is formed as a result of chromosomal aberrations: translocations (exchange of sites between two chromosomes), deletion (loss of the site of a chromosome), inversions (a revolution of the site of a chromosome) or amplification (formation of additional copies of the site of a chromosome). It is supposed that the genetic disorders causing development of a sharp limfoblastny leukosis arise in the pre-natal period, however for completion of process of formation of a malignant clone additional external circumstances quite often are required.
Among risk factors of developing of a sharp limfoblastny leukosis usually first of all specify beam influences: accommodation in a zone with the increased level of the ionizing radiation, radiotheraphy at treatment of other oncological diseases, numerous radiological researches, including in the pre-natal period. Communication level, and also validity of existence of dependence between various beam influences and development of a sharp limfoblastny leukosis strongly differ.
So, the interrelation between leukoses and radiation therapy is considered proved today. The risk of developing of a sharp limfoblastny leukosis after radiotheraphy makes 10%. The disease is diagnosed for 85% of patients within 10 years after the termination of a course of radiation therapy. Communication between radiological researches and development of a sharp limfoblastny leukosis remains at the level of assumptions now. The reliable statistical data confirming this theory do not exist yet.
Many researchers point to possible communication between OLL and infectious diseases. The virus of the causative agent of a sharp limfoblastny leukosis is not revealed yet. There are two main hypotheses. The first – OLL is called by one yet not established virus, however the disease arises only with predisposition. The second – different viruses can become the reason of development of a sharp limfoblastny leukosis, the risk of development of a leukosis in children increases at a lack of contacts with pathogenic microorganisms of early age (at "not fitness" of immune system). So far both hypotheses are not proved. Authentic data on existence of communication between leukoses and viral diseases are received only for T-cellular leukoses at the adult patients living in the countries of Asia.
The probability of development of a sharp limfoblastny leukosis increases at contact of mother with some toxic substances in the period of a gestation, at some genetic anomalies (Fankoni's anemia, a Down syndrome, Shvakhman's syndrome, Klaynfelter's syndrome, Viskotta-Aldrich's syndrome, a neurofibromatosis, a tseliakiya, the immune violations hereditarily caused), existence of oncological diseases in the family anamnesis and reception of tsitostatik. Some experts note possible negative impact of smoking.
Symptoms of a sharp limfoblastny leukosis
The disease develops promptly. By the time of diagnosis the total mass of limfoblast in an organism can make 3-4% of body lump that is caused by rough proliferation of cages of a malignant clone for 1-3 last months. Within a week the quantity of cages increases approximately twice. Distinguish several syndromes characteristic of a sharp limfoblastny leukosis: intoksikatsionny, hyper plastic, anemichesky, hemorrhagic, infectious.
The Intoksikatsionny syndrome includes weakness, fatigue, fever and loss of weight. Temperature increase can be provoked by both the main disease, and infectious complications which especially often develop in the presence of a neytropeniya. The hyper plastic syndrome at a sharp limfoblastny leukosis is shown by increase in lymph nodes, liver and spleen (as a result of leukemic infiltration of a parenchyma of bodies). At increase in parenchymatous bodies belly-aches can develop. Increase in volume of marrow, infiltration of a periosteum and fabrics of articulate capsules can become the reason of the aching bone and articulate pains.
Existence of an anemichesky syndrome is demonstrated by weakness, dizzinesses, pallor of skin and increase of warm reductions. Thrombocytopenia and thromboses of small vessels become the reason of development of a hemorrhagic syndrome at a sharp limfoblastny leukosis. On skin and mucous petekhiya and ekhimoza come to light. At bruises easily there are extensive hypodermic hemorrhages. The raised bleeding from wounds and scratches, hemorrhages in a retina, desnevy and nasal bleedings are observed. Some patients with a sharp limfoblastny leukosis have gastrointestinal bleedings which are followed by bloody vomiting and a tar-like chair.
Immune violations at a sharp limfoblastny leukosis are shown by frequent infection of wounds, scratches and traces from pricks. Various bacterial, viral and fungal infections can develop. At increase in lymph nodes of a sredosteniye the breath violations caused by reduction of volume of lungs are noted. Respiratory insufficiency is found at the T-cellular sharp limfoblastny leukosis more often. The neuroleukoses provoked by infiltration of covers back and a brain, are more often noted during the recurrence.
When involving TsNS positive meningealny symptoms and signs of increase in intra cranial pressure (hypostasis of disks of optic nerves, a headache, nausea and vomiting) come to light. Sometimes defeat of TsNS at a sharp limfoblastny leukosis proceeds asymptomatically and is diagnosed only after the research of tserebrospinalny liquid. 5-30% of boys have infiltrates in testicles. Patients of both floors on skin and mucous membranes can have crimson and cyanotic infiltrates (leykemid). Are in rare instances observed by vypotny perikardit also dysfunction of kidneys. Cases of damages of intestines are described.
Taking into account features of clinical symptomatology it is possible to allocate four periods of development of a sharp limfoblastny leukosis: initial, heat, remission, terminal. Duration of an initial stage makes 1-3 months. The nonspecific symptomatology prevails: slackness, fatigue, deterioration in appetite, also the increasing pallor of skin subfebrilitt. Headaches, belly-aches, bones and joints are possible. In the period of a heat of a sharp limfoblastny leukosis all listed above characteristic syndromes come to light. During remission of display of a disease disappear. The terminal period is characterized by the progressing deterioration in a condition of the patient and comes to the end with a lethal outcome.
Diagnosis of a sharp limfoblastny leukosis
The diagnosis is exposed taking into account clinical signs, results of the analysis of peripheral blood and data of a miyelogramma. In peripheral blood of patients with a sharp limfoblastny leukosis anemia, thrombocytopenia, increase in SOE and change of quantity of leukocytes come to light (usually – ). Limfoblasta make 15-20 and more percent from total of leukocytes. The quantity of neutrophils is reduced. In a miyelogramma blastny cages prevail, the expressed oppression of an eritroidny, neytrofilny and trombotsitarny sprout is defined.
At a sharp limfoblastny leukosis enter the program of inspection a lyumbalny puncture (for a neuroleukosis exception), ultrasonography of abdominal organs (for assessment of a condition of parenchymatous bodies and lymph nodes), a thorax X-ray analysis (for detection of the increased sredosteniye lymph nodes) and biochemical blood test (for identification of dysfunction of a liver and kidneys). The differential diagnosis of a sharp limfoblastny leukosis is carried out with other leukoses, poisonings, states at serious infectious diseases, an infectious limfotsitoz and an infectious mononukleoz.
Treatment and the forecast at a sharp limfoblastny leukosis
Basis of therapy are himiopreparata. Allocate two stages of treatment of OLL: a stage of intensive therapy and a stage of the supporting therapy. The stage of intensive therapy of a sharp limfoblastny leukosis includes two phases and about half a year lasts. In the first phase carry out intravenous polychemotherapy for achievement of remission. Blood formation normalization, existence no more than 5% of blast in marrow and lack of blast in peripheral blood testify to a condition of remission. In the second phase hold events for extension of remission, delay or the termination of proliferation of cages of a malignant clone. Introduction of medicines is also carried out intravenously.
Duration of a stage of the supporting therapy at a sharp limfoblastny leukosis makes about 2 years. During this period of the patient write out on out-patient treatment, appoint medicines for oral administration, carry out regular inspections for control over a condition of marrow and peripheral blood. The plan of treatment of a sharp limfoblastny leukosis is made individually taking into account risk level at the specific patient. Along with chemotherapy use immunochemotherapy, radiotheraphy and other techniques. At low efficiency of treatment and high risk of development of a recurrence perform transplantation of marrow. Average five-year survival at the V-cellular sharp limfoblastny leukosis at children's age makes 80-85%, in the adult – 35-40%. At T-limfoblastnom a leukosis the forecast is less favorable.