Leukemia – the malignant defeat of tissue of marrow leading to violation of maturing and a differentiation of the haematogenic cages predecessors of leukocytes, their uncontrollable growth and a dissemination on an organism in the form of leukemic infiltrates. As symptoms of leukemia weakness, weight loss, fever, bone pains, causeless gemorragiya, lymphadenitis, spleno-and a gepatomegaliya, meningealny symptoms, frequent infections can act. The diagnosis of leukemia is confirmed by the general blood test, a sternalny puncture with a marrow research, a trepanobiopsiya. Treatment of leukemia demands long continuous polychemotherapy, symptomatic therapy, if necessary - transplantations of marrow or stem cells.
Leukemia (leukosis, blood cancer, leukemia) – the tumoral disease of the haematogenic system () connected with replacement of healthy specialized cells of a leykotsitarny row by abnormally changed leucemic cages. Leukemia is characterized by bystry expansion and system defeat of an organism - the haematogenic and blood systems, lymph nodes and lymphoid educations, a spleen, a liver, TsNS, etc. Leukemia affects also adults and children, is the most often found oncological disease of children's age. Males are ill 1,5 times more often than women.
Leucemic cages are not capable to a full-fledged differentiation and performance of the functions, but at the same time have bigger life expectancy, high potential of division. Leukemia is followed by gradual replacement of populations of normal leukocytes (granulocytes, monocytes, lymphocytes) and their predecessors, and also deficiency of platelets and erythrocytes. It is promoted by active self-reproduction of leucemic cages, their higher sensitivity to growth factors, allocation of a growth factor of the tumor cells and factors oppressing normal blood formation.
Classification of leukemia
On features of development allocate sharp and chronic leukemia. At sharp leukemia (50-60% of all cases) there is a rapid progressing growth of population of the low-differentiated blastny cages which lost ability to maturing. Taking into account their morphological, cytochemical, immunological features sharp leukemia is subdivided into limfoblastny, miyeloblastny and undifferentiated forms.
The Sharp Limfoblastny Leukaemia (SLL) – makes up to 80-85% of cases of a leukosis at children, mainly, at the age of 2-5 years. The tumor is formed on the lymphoid line of blood formation and consists of predecessors of lymphocytes - limfoblast (L1, L2, L3 of the types) relating to a B-cellular, T-cellular or O-cellular proliferative sprout.
The Sharp Miyeloblastny Leukaemia (SML) – result of defeat of the myeloid line of blood formation; at the heart of leucemic growths – miyeloblasta and their descendants, other types of blastny cages. At children the share of OML makes 15% of all leukoses, progressive increase in frequency of a disease is noted with age. Differentiate several options of OML - with the minimum signs of a differentiation (M0), without maturing (M1), with signs of maturing (Sq.m), promiyelotsitarny (M3), miyelomonoblastny (M4), monoblastny (M5), eritroidny (M6) and megakariotsitarny (M7).
Undifferentiated leukemia is characterized by growth of early cages predecessors without signs of a differentiation presented by uniform small plyuripotentny stem cells of blood or partially determined semi-stem cells.
The chronic form of leukemia is fixed in 40-50% of cases, is most widespread among adult population (40-50 years and are more senior), especially among the persons which underwent the ionizing radiation. Chronic leukemia develops slowly, within several years, is shown by the excess growth of number of mature, but functionally inactive, long-living leukocytes – B and T-lymphocytes at a lymphocytic form (HLL) and granulocytes and the ripening cages predecessors of a myeloid row at a miyelotsitarny form (HML). Allocate separately juvenile, children's and adult options of HML, an eritremiya, a miyelomny disease (plazmotsity). Eritremiya is characterized by leucemic transformation of erythrocytes, a high neytrofilny leykotsitoz and trombotsitozy. A source of a miyelomny disease is the tumoral growth of plazmotsit, violations of exchange of Ig.
Act as the reason of leukemia inside - and interchromosomal abberation – violations of molecular structure or exchange of sites of chromosomes (a deletion, inversion, fragmentation and a translocation). For example, at chronic myeloid leukemia emergence of the Philadelphian chromosome with t translocation is observed (9; 22). Leucemic cages can arise at any stage of a hematopoiesis. At the same time chromosomal violations can be primary - with change of properties of the haematogenic cage and creation of its specific clone (monoclonal leukemia) or secondary, arising in the course of proliferation of genetically unstable leucemic clone (more malignant polyclonal form).
Leukemia comes to light at patients with chromosomal diseases (a Down syndrome, Klaynfelter's syndrome), primary immunodeficiency more often. It is possible to refer infection with onkogenny viruses to the possible reason of leukemia. The disease is promoted by existence of hereditary predisposition as it meets in the families having patients with leukemia more often.
Malignant transformation of the haematogenic cages can happen under the influence of various mutagen factors: ionizing radiation, electromagnetic field of high voltage, chemical carcinogens (drugs, pesticides, cigarette smoke). Secondary leukemia is often connected with carrying out beam or chemotherapy in the course of treatment of other oncopathology.
The course of leukemia passes several stages: initial, the developed manifestations, remission, recovery, a recurrence and terminal. Symptoms of leukemia are not specific and have common features at all types of a disease. They are defined by a tumoral giperplaziya and infiltration of marrow, blood and lymphatic systems, TsNS and various bodies; deficiency of normal blood cells; hypoxia and intoxication, development of hemorrhagic, immune and infectious consequences. Extent of display of leukemia depends on localization and massiveness of leucemic damage of a hematopoiesis, fabrics and bodies.
At sharp leukemia the general malaise, weakness, a loss of appetite and weight loss, pallor of skin quickly appear and accrue. Patients are disturbed by high temperature (39-40 °C), oznoba, artralgiya and bone pains; easily arising bleeding mucous, skin hemorrhages (petekhiya, bruises) and bleedings of different localization.
There is an increase in regionarny lymph nodes (cervical, axillary, inguinal), swelling of salivary glands, is observed a gepatomegaliya and a splenomegaliya. Often infectious and inflammatory processes of a mucous rotoglotka, steady against treatment, - stomatitis develop, gingivit, ulcer and necrotic quinsy. Anemia, comes to light, the DVS-syndrome can develop.
For neuroleukaemia meningealny symptoms (vomiting, severe headaches, hypostasis of an optic nerve, a spasm), backbone pains, paresis, paralysis are indicative. At OLL massive blastny defeats of all groups of lymph nodes, a thymus, lungs, a sredosteniye, a GIT, kidneys, genitals develop; at OML - multiple miyelosarkoma (hloroma) in a periosteum, internals, fatty cellulose, on skin. At elderly patients at leukemia stenocardia, violation of a rhythm of heart are possible.
Chronic leukemia has slowly or moderately progressing current (from 4-6 to 8-12 years); typical displays of a disease are observed in the developed stage (accelerations) and terminal (blastny crisis) when there is metastasis of blastny cages out of marrow limits. Against the background of an aggravation of the general symptoms sharp exhaustion, increase in the sizes of internals, especially spleens, generalized lymphadenitis, pustulous damages of skin (piodermiya), pneumonia is allocated.
In case of an eritremiya vascular thromboses of the lower extremities, brain and coronary arteries develop. The Miyelomny disease proceeds with single or multiple tumoral infiltrates of bones of a skull, backbone, edges, a shoulder, hip; the osteolysis and osteoporosis, bone deformation and frequent changes which are followed by a pain syndrome. Sometimes the miyelomny nephropathy with HPN develops AL .
Death of the patient with leukemia can occur at any stage because of extensive gemorragiya, hemorrhages in vitals, a rupture of a spleen, development is purulent - septic complications (peritonitis, sepsis), severe intoxication, renal and heart failure.
Diagnosis of leukemia
Within diagnostic testings at leukemia the general and biochemical blood test, a diagnostic puncture of marrow (sternalny) and a spinal cord (lyumbalny), a trepanobiopsiya and a biopsy of lymph nodes, a X-ray analysis, ultrasonography, KT and MPT of vitals are made.
In peripheral blood the expressed anemia, thrombocytopenia, change of total number of leukocytes (usually increase, but also the shortcoming is possible), violation of a leykotsitarny formula, existence of atypical cages are observed. At sharp leukemia blasta and small percent of mature cages without transitional elements ("a leukemic failure") are defined, at chronic – marrowy cages of different classes of development.
At leukemia the research of bioptat of marrow (miyelogramm) and tserebrospinalny liquid including morphological, cytogenetic, cytochemical and immunological analyses is key. It allows to specify forms and subtypes of leukemia that is important for the choice of the protocol of treatment and forecasting of a disease. At sharp leukemia the level of undifferentiated blast in marrow makes more than 25%. Important criterion is detection of the Philadelphian chromosome (Ph-chromosome).
Leucemic infiltration of internals is established on ultrasonography of lymph nodes, abdominal cavity and small pelvis, X-ray analysis of a thorax, skull, bones and joints, KT of a thorax, MRT of a head and spinal cord with contrasting, EhoKG. At leukemia consultation of the otolaryngologist, neurologist, urologist, ophthalmologist is shown.
Leukemia is differentiated from autoimmune trombotsitopenichesky by purples, neuroblastomas, juvenile rheumatoid arthritis, an infectious mononukleoz, and also other tumoral and infectious diseases causing leykemoidny reaction.
Treatment of leukemia
Treatment of leukemia is carried out by hematologists in specialized oncohematological clinics according to the adopted protocols, with observance of accurately established periods, the main stages and volumes of medical and diagnostic measures for each form of a disease. The purpose of treatment of leukemia – receiving long full kliniko-hematologic remission, restoration of normal blood formation and the prevention of a recurrence, and at an opportunity – full treatment of the patient.
Sharp leukemia demands the immediate beginning of an intensive course of treatment. As a basic method at leukemia the multicomponent chemotherapy to which sharp forms (efficiency are most sensitive at OLL – 95%, OML ~ 80%) and leukoses of children's age (up to 10 years) is applied. Combinations of various cytostatic medicines are applied to achievement of remission of sharp leukemia at the expense of a reduction and an eradikation of leucemic cages. During remission treatment in the form of the chemotherapy fixing (consolidating), and then supporting with addition in schemes of new tsitostatik continues long (within several years). For prevention of neuroleukaemia during remission intratekalny and intralyumbalny local introduction of himiopreparat and radiation of a brain is shown.
Treatment of OML is problematic because of frequent development of complications of hemorrhagic and infectious character. The promiyelotsitarny form of leukemia which turns into full kliniko-hematologic remission under the influence of stimulators of a differentiation of promiyelotsit is more favorable. In a stage of full remission of OML the allogenny transplantation of marrow (or introduction of stem cells) allowing to reach in 55-70% of cases 5 summer survival without recurrence is effective.
At chronic leukemia in a preclinical stage of rather constant observation and the all-strengthening actions (a full-fledged diet, a rational work-rest schedule, an exception of insolations, physiotherapy). Out of an exacerbation of chronic leukemia the substances blocking tirozinkinazny activity of protein of Bcr-Abl are appointed; but they are less effective in a phase of acceleration and blastny crisis. In the first year of a disease a-interferon introduction is expedient. At HML allogenny transplantation of marrow from the related or unrelated HLA donor can yield good results (60% of cases of full remission within 5 and more years). At an aggravation it is urgently appointed mono - or polychemotherapy. Application of radiation of lymph nodes, spleens, skin is possible; and according to certain indications – carrying out a splenektomiya.
As symptomatic measures at all forms of leukemia haemo static and dezintoksikatsionny therapy, infusions of trombotsitarny and leykotsitarny weight, antibiotic treatment are applied.
Forecast of leukemia
The forecast of leukemia depends forms of a disease, prevalence of defeat, risk group of the patient, terms of diagnosis, the response to treatment, etc. Leukemia has worse forecast at male patients, at children 10 years are aged more senior and 60 years are more senior than adults; at the high level of leukocytes, existence of the Philadelphian chromosome, neuroleukaemia; in cases of overdue diagnostics. Sharp leukemias have much the worst forecast because of a prompt current and in the absence of treatment quickly lead to death. At children at timely and rational treatment the forecast of sharp leukemia is more favorable, than at adults. The good forecast of leukemia the probability of 70% 5 summer survival and more is considered; risk of a recurrence - less than 25%.
Chronic leukemia at achievement of blastny crisis gets an aggressive current with risk of a lethal outcome because of development of complications. At the correct treatment of a chronic form it is possible to achieve remission of leukemia for many years.