Sharp leukosis – tumoral defeat of the haematogenic system as which morphological basis the unripe (blastny) cages which are forcing out normal haemo poetic sprouts act. The clinical symptomatology of a sharp leukosis is presented by the progressing weakness, unmotivated rise in temperature, artralgiya and ossalgiya, bleedings of various localizations, a limfadenopatiya, a gepatosplenomegaliya, gingivita, stomatitises, quinsies. Confirmation of the diagnosis requires a research of a gemogramma, punktat of marrow, a bioptat of a podvzdoshny bone and lymph nodes. The basis of treatment of sharp leukoses is made by chemotherapeutic courses and accompanying therapy.
Sharp leukosis - a form of leukemia at which normal marrowy blood formation is forced out by a little differentiated cages predecessors of leukocytes with their subsequent accumulation in peripheral blood, infiltration of fabrics and bodies. The terms "sharp leukosis" and "chronic leukosis" reflect not only duration of a course of a disease, but also the morphological and cytochemical characteristic of tumor cells. The sharp leukosis is the most frequent form of gemoblastoz: it develops at 3-5 of 100 thousand people; a ratio of adults and children – 3:1. At the same time at persons 40 years are more senior statistically more often the sharp myeloid leukosis is diagnosed, and children have a sharp limfoblastny leukosis.
Reasons of a sharp leukosis
The prime cause of a sharp leukosis is the mutation of the haemo poetic cage giving rise to a tumoral clone. The mutation of the haematogenic cage leads to violation of its differentiation at an early stage of unripe (blastny) forms with further proliferation of the last. The formed tumor cells replace normal sprouts of a hematopoiesis in marrow, and further leave in blood and are carried in various fabrics and bodies, causing their leukemic infiltration. All blastny cages bear in themselves identical morphological and cytochemical features that testifies in favor of their klonalny origin from one cage ancestor.
The reasons starting mutational process are not known. In hematology it is accepted to speak about the risk factors increasing probability of development of a sharp leukosis. First of all, it is genetic predisposition: existence in a family of patients with a sharp leukosis practically triples risk of a disease at close relatives. The risk of developing of a sharp leukosis increases at some chromosomal anomalies and genetic pathologies - a Down syndrome, Klaynfelter, Viskotta-Aldrich and Louis-Barre's syndrome, Fankoni's anemia, etc.
It is probable that activation of genetic predisposition happens under the influence of various exogenous factors. Among the last the ionizing radiation, chemical carcinogens (benzene, arsenic, toluene, etc.), the cytostatic medicines used in oncology can act. Quite often sharp leukosis turns out to be consequence of antineoplastic therapy of other gemoblastoz – a limfogranulematoza, nekhodzhkinsky lymphoma, a miyelomny disease. Communication of sharp leukoses with the previous viral infections oppressing immune system is noticed; the accompanying hematologic diseases (some forms of anemias, miyelodisplaziya, a paroksizmalny night gemoglobinuriya, etc.).
Classification of a sharp leukosis
In haemato-oncology the international FAB classification of sharp leukoses differentiating various forms of a disease depending on morphology of tumor cells on limfoblastny (caused by the low-differentiated predecessors of lymphocytes) and nelimfoblastny (the uniting other forms) is standard.
1. Sharp limfoblastny leukoses of adults and children ( - In - a form, the V-form, , the T-form, neither T nor the V-form).
2. Sharp nelimfoblastny (myeloid) leukoses:
- the lake miyeloblastny (it is caused by nekontrorliruyemy proliferation of predecessors of granulocytes)
- the lake mono - and the lake miyelomonoblastny (are characterized by the strengthened reproduction of monoblast)
- the lake megakarioblastny (it is connected with prevalence of undifferentiated megakariotsit – predecessors of platelets)
- the lake eritroblastny (it is caused by proliferation of eritroblast)
3. Sharp undifferentiated leukosis.
The course of sharp leukoses passes a number of stages:
I (initial) - the general nonspecific symptoms prevail
II (developed) - it is characterized by accurately expressed clinical and hematologic symptoms of a gemoblastoz. Includes:
- debut or first "attack"
- incomplete or full remission
- recurrence or recovery
III (terminal) – it is characterized by deep oppression of a normal hematopoiesis.
Symptoms of a sharp leukosis
The demonstration of a sharp leukosis can be sudden or erased. The beginning which is characterized by high fever, intoxication, perspiration, sharp decline of forces, anorexia is typical. During first "attack" patients note persistent muscle and bones pains, artralgiya. Sometimes the initial stage of a sharp leukosis masks under a SARS or quinsy; can be the first symptoms of leukemia ulcer stomatitis or hyper plastic gingivit. Quite often the disease is found incidentally at a preventive research of a gemogramma or is retrospective when the sharp leukosis passes into the following stage.
In the developed period of a sharp leukosis anemichesky, hemorrhagic, intoksikatsionny and hyper plastic syndromes develop.
Anemichesky manifestations are caused by violation of synthesis of erythrocytes, on the one hand, and the raised bleeding – with another. They include pallor of integuments and mucous, constant fatigue, dizziness, heartbeat, the raised hair loss and fragility of nails, etc. Expressiveness of tumoral intoxication amplifies. In the conditions of an absolute leykopeniya and decline of immunity various infections easily join: pneumonia, candidiasis, pyelonephritis etc.
The expressed thrombocytopenia is the cornerstone of a hemorrhagic syndrome. Range of hemorrhagic manifestations fluctuates from small single petekhiya and bruises to a gematuriya, desnevy, nasal, uterine, gastrointestinal bleedings and so forth. In process of progressing of a sharp leukosis of bleeding there can be all massivny owing to development of the DVS-syndrome.
The hyper plastic syndrome is connected with leukemic infiltration of both marrow, and other bodies. At patients with a sharp leukosis increase in lymph nodes (peripheral, mediastinalny, intra belly), a hypertrophy of almonds, a gepatosplenomegaliya is observed. There can be leucemic infiltrates of skin (leykemida), brain covers (neuroleukosis), damage of lungs, a myocardium, kidneys, ovaries, testicles, etc. bodies.
Full kliniko-hematologic remission is characterized by lack of the extra marrowy leukemic centers and the maintenance of blast in a miyelogramma less than 5% (incomplete remission – less than 20%). Lack of kliniko-hematologic manifestations within 5 years is regarded as recovery. In case of increase in blastny cages in marrow more than 20%, their emergence in peripheral blood, and also identifications of the extra marrowy metastatic centers are diagnosed a recurrence of a sharp leukosis.
The terminal stage of a sharp leukosis is stated at inefficiency of chemotherapeutic treatment and impossibility of achievement of kliniko-hematologic remission. Progressing of tumoral growth, development of malfunction incompatible with life of internals are signs of this stage. Hemolytic anemia, repeated pneumonia, piodermiya, abscesses and phlegmons of soft fabrics, sepsis, the progressing intoxication join the described clinical manifestations. Not stopped bleedings, cerebral hemorrhages, infectious and septic complications become a cause of death of patients.
Diagnosis of a sharp leukosis
Assessment of morphology of cells of peripheral blood and marrow is at the head of diagnosis of sharp leukoses. Anemia, thrombocytopenia, high SOE, (the leykopeniye is more rare), presence of blastny cages is characteristic of a gemogramma at leukemia. The phenomenon of "a leukemic gaping" is indicative - intermediate stages between blasta and mature cages are absent.
For the purpose of confirmation and identification of a kind of a sharp leukosis the sternalny puncture with a morphological, cytochemical and immunophenotypical research of marrow is carried out. At a research of a miyelogramma increase in percent of blastny cages (from 5% and above), , oppression of a red sprout of blood formation (except cases of the lake of an eritromiyeloz) and absolute decrease or lack of megakariotsit pays attention (except cases of the lake of a megakarioblastny leukosis). Cytochemical marker reactions and immunophenotyping of blastny cages allow to establish a form of a sharp leukosis precisely. At ambiguity of interpretation of the analysis of marrow resort to carrying out a trepanobiopsiya.
For the purpose of an exception of leukemic infiltration of internals the spinal puncture with a likvor research, a X-ray analysis of a skull and bodies of a thorax, ultrasonography of lymph nodes, liver and spleen is made. Except the hematologist, patients with a sharp leukosis have to be examined by the neurologist, the ophthalmologist, the otolaryngologist, the stomatologist. For assessment of weight of system violations the research of a koagulogramma, biochemical blood test, carrying out an electrocardiography, EhoKG, etc. can be required.
Differential and diagnostic actions are directed to an exception of HIV infection, an infectious mononukleoz, a Cytomegaloviral infection, kollagenoz, trombotsitopenichesky purples, an agranulotsitoz; pan-cytosinging at aplastic anemia, B12 and foliyevodefitsitny anemia; leykemoidny reactions at whooping cough, tuberculosis, sepsis, etc. diseases.
Treatment of a sharp leukosis
Patients with a sharp leukosis are treated in hospitals of an oncohematological profile. In chambers the strengthened sanitary and disinfection mode will be organized. Patients with a sharp leukosis need carrying out hygienic processing of an oral cavity, prevention of decubituses, a toilet of genitals after physiological departures; the organizations of the high-calorific and vitaminized food.
Directly treatment of sharp leukoses is carried out consistently; the main stages of therapy include achievement (induction) of remission, its fixing (consolidation) and maintenance, prevention of complications. The standardized schemes of polychemotherapy which are selected the hematologist taking into account a morphological and cytochemical form of a sharp leukosis are for this purpose developed and are used.
At a favorable situation remission is usually reached within 4-6 weeks of the strengthened therapy. Then, within consolidation of remission 2-3 more courses of polychemotherapy are conducted. The supporting antirecurrent therapy is performed within not less than 3 years. Along with chemotherapy at a sharp leukosis performing the accompanying treatment directed to the prevention of an agranulotsitoz, thrombocytopenia, the DVS-syndrome, infectious complications, neuroleukaemias (antibiotic treatment, transfusion of eritrotsitarny, trombotsitarny weight and freshly frozen plasma, endolyumbalny introduction of tsitostatik) is necessary. At leukemic infiltration of a throat, a sredosteniye, testicles, etc. bodies X-ray therapy of the centers of defeat is carried out.
In case of successful treatment destruction of a clone of leucemic cages, blood formation normalization is reached that promotes induction of the long bezretsidivny period and recovery. For the prevention of a recurrence of a sharp leukosis transplantation of marrow after preliminary conditioning by chemotherapy and total radiation can be carried out.
According to the available statistical data, use of modern cytostatic means leads to transition of a sharp leukosis to a remission phase at 60-80% of patients; from them it is possible to achieve an absolute recovery from 20-30%. In general the forecast at sharp limfoblastny leukoses is more favorable, than at miyeloblastny.