Leukosis at children – the malignant disease of blood which is characterized by tumoral proliferation of unripe cages predecessors of leukocytes. Clinical displays of a leukosis at children can include increase in lymph nodes, a hemorrhagic syndrome, bone and joints pains, a gepatosplenomegaliya, defeat of TsNS, etc. Diagnosis of a leukosis at children is promoted by the developed general blood test, carrying out a sternalny puncture with a research of a punktat of marrow. Treatment of a leukosis at children is carried out in specialized hematologic hospitals by means of chemotherapy, an immunotherapy, replacement therapy, transplantation of marrow.
Leukosis at children
Leukosis at children (leukemia) – system , followed by violation of marrowy blood formation and replacement of normal blood cells with unripe blastny cages of a leykotsitarny row. In children's haemato-oncology the frequency of leukoses makes 4-5 cases on 100 thousand children. Statistically, the sharp leukosis is the most frequent oncological disease of children's age (about 30%); most often the blood cancer affects children at the age of 2-5 years. As an urgent problem of pediatrics serves the tendency to growth of incidence of leukoses observed in recent years among children and the remaining high lethality.
The leukosis reasons at children
Some aspects of development of a leukosis in children remain not clear so far. At the present stage etiologichesky influence of radiation, onkogenny virus strains, chemical factors is proved, to hereditary predisposition, endogenous violations (hormonal, immune) on the frequency of developing of a leukosis at children. The secondary leukosis can develop at the child who transferred in the anamnesis beam or chemotherapy concerning other oncological disease.
On today's put mechanisms of development of a leukosis in children it is accepted to consider from the point of view of the mutational theory and the clonal concept. The mutation of DNA of the haematogenic cage is followed by failure of a differentiation at a stage of an unripe blastny cage with the subsequent proliferation. Thus, leucemic cages – are no other than clones of the mutating cage incapable of a differentiation and maturing and overwhelming normal sprouts of blood formation. Getting to blood, blastny cages are carried on all organism, promoting leukemic infiltration of fabrics and bodies. Metastatic penetration of blastny cages through a hematoencephalic barrier leads to infiltration of covers and substances of a brain and to development of a neuroleukosis.
It is noted that at children with a Down syndrome the leukosis develops in 15 times more often than at other children. The increased risk of development of a leukosis and other tumors is available for children with syndromes Li-Fraumeni, Klaynfelter, Viskott-Aldrich, Blum, Fankoni's anemia, primary immunodeficiencies (H-linked by an agammaglobulinemiya, Louis-Barre's ataxy-teleangiektaziyey, etc.), a politsitemiya, etc.
Classification of a leukosis at children
On the basis of duration of a disease allocate sharp (up to 2 years) and chronic (more than 2 years) leukosis forms at children. Sharp leukoses occur at children in absolute majority of cases (97%). The special form of a sharp leukosis at children is represented by a congenital leukosis.
Taking into account these morphological characteristics of tumor cells sharp leukoses at children share on limfoblastny and nelimfoblastny. The Limfoblastny leukosis develops at uncontrolled proliferation of unripe lymphocytes - limfoblast and can be three types: L1 - with small limfoblasta; L2 – with big polymorphic limfoblasta; L3 - with big polymorphic limfoblasta from vakuolizatsiy cytoplasm. On anti-gene markers distinguish 0-cellular (70-80%), T-cellular (15-25%) and V-cellular (1-3%) sharp limfoblastny leukoses at children. Among sharp limfoblastny leukoses at children the leukosis meets with cages of the L1 type more often.
Among nelimfoblastny leukoses, depending on prevalence of these or those blastny cages, distinguish miyeloblastny low-differentiated (M1), miyeloblastny the high-differentiated (Sq.m), promiyelotsitarny (M3), miyelomonoblastny (M4), monoblastny (M5), (M6), megakariotsitarny (M7), eozinofilny (M8), an undifferentiated (M0) leukosis at children.
In the clinical course of a leukosis at children allocate 3 stages taking into account which medical tactics is built.
- I – a sharp phase of a leukosis at children; covers the period from a demonstration of symptoms before improvement of kliniko-hematologic indicators as a result of the carried-out therapy;
- II – incomplete or full remission. At incomplete remission normalization of a gemogramma and clinical indicators is noted; the quantity of blastny cages in a punktata of marrow makes no more than 20%. Full remission is characterized by existence in a miyelogramma no more than 5% of blastny cages;
- III - a leukosis recurrence at children. Against the background of hematologic wellbeing the ekstramedullyarny centers of leucemic infiltration in the nervous system, testicles easy, etc. bodies appear.
Leukosis symptoms at children
In most cases the clinic of a leukosis develops gradually and is characterized by nonspecific symptoms: fatigue of the child, sleep disorder, loss of appetite, ossalgiya and artralgiya, unmotivated temperature increase of a body. Sometimes the leukosis at children demonstrates suddenly from an intoksikatsionny or hemorrhagic syndrome.
At the children having a leukosis the expressed pallor of integuments and mucous membranes is noted; sometimes skin gets an icteric or earthy shade. Owing to leukemic infiltration of mucous membranes at children quite often arise gingivit, stomatitis, tonsillitis. The leukemic giperplaziya of lymph nodes is shown by a limfadenopatiya; salivary glands – a sialadenopatiya; a liver and a spleen – a gepatosplenomegaliya.
The hemorrhagic syndrome which is characterized by hemorrhages in skin and mucous, a gematuriya, nasal, uterine, gastrointestinal, pulmonary bleedings, hemorrhages in a cavity of joints, etc. is typical for the course of a sharp leukosis at children. The natural satellite of a sharp leukosis at children is the anemichesky syndrome caused by oppression of an eritropoez and bleedings. Expressiveness of anemia at children depends on extent of proliferation of blastny cages in marrow.
Cardiovascular frustration at leukoses at children can be expressed by development of tachycardia, arrhythmia, expansion of borders of heart (according to a X-ray analysis of bodies of a thorax), diffusion changes of a myocardium (according to the ECG), decrease in fraction of emission (according to EhoKG).
The Intoksikatsionny syndrome accompanying the course of a leukosis at children proceeds with considerable weakness, fever, perspiration, anorexia, nausea and vomiting, a hypotrophy. As manifestations of an immunodeficient syndrome at a leukosis at children serves stratification of infectious and inflammatory processes which can accept the heavy, menacing current. Death of the children having a leukosis quite often happens owing to heavy pneumonia or sepsis.
Extremely dangerous complication of a leukosis at children is leukemic infiltration of a brain, brain covers and nervous trunks. The neuroleukosis is followed by dizziness, a headache, nausea, a diplopiya, a rigidnost of occipital muscles. At infiltration of substance of a spinal cord development of paraparesis of legs, violations of sensitivity, pelvic frustration is possible.
Diagnosis of a leukosis at children
The leading role in primary detection of leukoses at children belongs to the pediatrician; further inspection and maintaining the child is carried out to nurseries onkogematology. The basis of diagnosis of leukoses at children is made by laboratory methods: research of peripheral blood and marrow.
At a sharp leukosis at children characteristic changes in the general blood test come to light: anemia; thrombocytopenia, retikulotsitopeniye, high SOE; various degree or leykopeniye (seldom), blastemiya, disappearance of basophiles and eosinophils. The phenomenon of "a leukemic failure" - lack of intermediate forms (young, stab, segmentoyaderny leukocytes) between mature and blastny cages is a typical sign.
The Sternalny puncture and research of a miyelogramma are obligatory in diagnosis of a leukosis at children. As a decisive argument in favor of a disease serves the maintenance of blastny cages from 30% and above. In the absence of accurate data for a leukosis at children by results of a marrow research, resort to a trepanobiopsiya (a puncture of a podvzdoshny bone). For definition of various options of a sharp leukosis at children cytochemical, immunological and cytogenetic researches are carried out. For the purpose of confirmation of the diagnosis of a neuroleukosis consultation of the children's neurologist and children's ophthalmologist, a lyumbalny puncture and a research of tserebrospinalny liquid, a skull X-ray analysis, an oftalmoskopiya is held.
Ultrasonography of salivary glands, ultrasonography of a liver and spleen, ultrasonography of a scrotum at boys, a X-ray analysis of bodies of a thorax, KT at children have auxiliary diagnostic value ultrasonography of lymph nodes (for identification of metastasises in various anatomic regions). Differential diagnosis of leukoses at children should be carried out with the leykozopodobny reaction observed at severe forms of tuberculosis, whooping cough, an infectious mononukleoz, a Cytomegaloviral infection, sepsis and having reversible tranzitorny character.
Treatment of a leukosis at children
Children with leukoses are hospitalized in specialized institutions of an oncohematological profile. For prevention of infectious complications the child is located to the separate box in which conditions are most approximate to sterile. Much attention is paid to food which has to be full and balanced.
The basis of treatment of leukoses at children is made by the polychemotherapy directed to a full eradikation of a leucemic clone. The protocols of treatment used at sharp limfoblastny and miyeloblastny leukoses differ with a combination of himiopreparat, their doses and ways of introduction. Stage-by-stage treatment of a sharp leukosis at children assumes achievement of kliniko-hematologic remission, its consolidation (fixing) supporting therapy, prevention or treatment of complications.
In addition to chemotherapy the active and passive immunotherapy can be carried out: introduction of leucemic cages, vaccines BTsZh, protivoospenny vaccine, interferon, immune lymphocytes, etc. As perspective methods of treatment of a leukosis at children serves transplantation of marrow, umbilical blood, stem cells.
Symptomatic therapy at leukoses at children includes transfusion of eritrotsitarny and trombotsitarny weight, performing haemo static therapy, antibiotic treatment of infectious complications, dezintoksikatsionny actions (intravenous infusions, haemo sorption, a plazmosorbtion, a plasma exchange).
The forecast of a leukosis at children
The prospects of development of a disease are defined by many factors: age of developing of a leukosis, cytoimmunological option, diagnosing stage etc. The worst forecast should be expected at the children who got sick with a sharp leukosis aged up to 2 years and is more senior than 10 years; having a limfadenopatiya and a gepatosplenomegaliya, and also a neuroleukosis at the time of establishment of the diagnosis; T - and V-cellular options of a leukosis, blastny . Predictively as favorable factors serve the sharp limfoblastny leukosis of L1 of type, an early initiation of treatment, bystry achievement of remission, age of children from 2 to 10 years. At girls with a sharp limfoblastny leukosis probability treatment is slightly higher, than at boys.
Lack of specific treatment of a leukosis at children is followed by a 100% lethality. Against the background of modern chemotherapy the five-year bezretsidivny course of a leukosis is noted at 50-80% of children. It is possible to speak about probable recovery after 6-7 years of lack of a recurrence. In order to avoid provocation of a recurrence physiotherapeutic treatment, change of climatic conditions is not recommended to children. Vaccinal prevention is carried out according to an individual calendar taking into account an epidemic situation.