Chronic myeloid leukemia
Chronic myeloid leukemia – the malignant miyeloproliferativny disease which is characterized by primary defeat of a granulotsitarny sprout. Long time asymptomatically can proceed. It is shown by tendency to a subfebrilitet, feeling of completeness in a stomach, frequent infections and increase in a spleen. The anemia and change of level of platelets which are followed by weakness, pallor and the raised bleeding are observed. At a final stage fever, a limfoadenopatiya and skin rash develop. The diagnosis is established taking into account the anamnesis, a clinical picture and these laboratory researches. Treatment – chemotherapy, radiotheraphy, bone marrow transplantation.
Chronic myeloid leukemia
Chronic myeloid leukemia – the oncological disease resulting from a chromosomal mutation with defeat of polipotentny stem cells and the subsequent uncontrollable proliferation of mature granulocytes. Makes 15% of total of gemoblastoz at adults and 9% of total number of leukoses in all age groups. Usually develops after 30 years, the peak of incidence of chronic myeloid leukemia is the share of age of 45-55 years. Children up to 10 years suffer exclusively seldom.
Chronic myeloid leukemia is equally widespread among women and among men. Because of an asymptomatic or malosimptomny current can become a casual find at a research of the blood test taken in connection with other disease or during routine inspection. At a part of patients chronic myeloid leukemia comes to light at final stages that limits possibilities of therapy and worsens survival indicators. Treatment is carried out by experts in the field of oncology and hematology.
Etiology and pathogenesis of chronic myeloid leukemia
Chronic myeloid leukemia is considered the first disease at which connection between development of pathology and a certain genetic disorder is authentically established. In 95% of cases the chromosomal translocation known as "the Philadelphian chromosome" is the confirmed reason of chronic myeloid leukemia. The essence of a translocation consists in mutual replacement of sites 9 and 22 of chromosomes. As a result of such replacement the steady open frame of reading is formed. Formation of a frame causes acceleration of cell fission and suppresses DNA restoration mechanism that increases probability of emergence of other genetic anomalies.
Among the possible factors promoting emergence of the Philadelphian chromosome in patients with chronic myeloid leukemia call the ionizing radiation and contact with some chemical compounds. The strengthened proliferation of polipotentny stem cells becomes a result of a mutation. At chronic myeloid leukemia proliferirut mainly mature granulocytes, but the abnormal clone includes also other blood cells: erythrocytes, monocytes, megakariotsita, are more rare – In - and T-lifotsity. Usual haemo poetic cages at the same time do not disappear and after suppression of an abnormal clone can form a basis for normal proliferation of blood cages.
The phasic current is characteristic of chronic myeloid leukemia. At the first, chronic (inactive) phase gradual aggravation of pathological changes at preservation of a satisfactory general condition is noted. In the second phase of chronic myeloid leukemia – a phase of acceleration of change become obvious, the progressing anemia and thrombocytopenia develop. A final stage of chronic myeloid leukemia is the blastny crisis which is followed by bystry ekstramedullyarny proliferation of blastny cages. Lymph nodes, bones, skin, TsNS etc. become a source of blast. In a phase of blastny crisis the condition of the patient with chronic myeloid leukemia sharply worsens, the heavy complications which are coming to the end with death of the patient develop. At some patients the phase of acceleration is absent, the chronic phase is replaced by blastny crisis at once.
Symptoms of chronic myeloid leukemia
The clinical picture is defined by a disease stage. The chronic phase on average proceeds 2-3 years, in certain cases – up to 10 years. The asymptomatic current or gradual emergence of "easy" symptoms is characteristic of this phase of chronic myeloid leukemia: weaknesses, some indisposition, decrease in working capacity and feeling of overflow of a stomach. At objective survey of the patient with chronic myeloid leukemia increase in a spleen can be found. On blood tests increase in quantity of granulocytes to 50-200 thousand / comes to light at the asymptomatic course of a disease and to 200-1000 thousand / at "easy" signs.
At initial stages of chronic myeloid leukemia perhaps some decrease in level of hemoglobin. In the subsequent normokhromny normotsitarny anemia develops. At a research of dab of blood of patients with chronic myeloid leukemia prevalence of young forms of granulocytes is noted: miyelotsit, promiyelotsit, miyeloblast. Deviations from the normal level of granularity in this or that party are observed (plentiful or very poor). Cytoplasm of cages unripe, bazofilny. Is defined . In the absence of treatment the chronic phase passes into an acceleration phase.
Both change of laboratory indicators, and deterioration in a condition of patients can testify to the beginning of a phase of acceleration of chronic myeloid leukemia. Weakness increase, increase in a liver and the progressing increase in a spleen is possible. At patients with chronic myeloid leukemia clinical symptoms of anemia and thrombocytopenia or a trobotsitoz come to light: pallor, bystry fatigue, dizzinesses, petekhiya, hemorrhages, the raised bleeding. Despite the carried-out treatment, in blood of patients with chronic myeloid leukemia the quantity of leukocytes gradually increases. At the same time increase of level of metamiyelotsit and miyelotsit is noted, emergence of single blastny cages is possible.
Blastny crisis is followed by sharp deterioration in a condition of the patient with chronic myeloid leukemia. There are new chromosomal anomalies, the monoclonal new growth is transformed to polyclonal. Increase of a cellular atipizm at oppression of normal sprouts of blood formation is noted. Pronounced anemia and thrombocytopenia are observed. The total quantity of blast and promiyelotsit in peripheral blood makes more than 30%, in marrow – more than 50%. Patients with chronic myeloid leukemia lose the weight and appetite. There are ekstramedullyarny centers of unripe cages (hloroma). Bleedings and heavy infectious complications develop.
Diagnosis of chronic myeloid leukemia
The diagnosis is established on the basis of a clinical picture and results of laboratory researches. The first suspicion of chronic myeloid leukemia often arises at increase in level of granulocytes in the general blood test appointed as routine inspection or inspection in connection with other disease. For specification of the diagnosis data of a histologic research of the material received at a sternalny puncture of marrow, however the final diagnosis can be used "chronic myeloid leukemia" is exposed at identification of the Philadelphian chromosome by means of PTsR, fluorescent hybridization or a cytogenetic research.
The question of a possibility of diagnosis chronic myeloid leukemia in the absence of the Philadelphian chromosome remains diskutabelny. Many researchers consider that similar cases can speak complex chromosomal violations because of which identification of this translocation becomes difficult. In some cases the Philadelphian chromosome can be found when using PTsR with the return transcription. At negative results of a research and the atypical course of a disease usually speak not about chronic myeloid leukemia, and about undifferentiated miyeloproliferativnom/miyelodisplastichesky frustration.
Treatment of chronic myeloid leukemia
Tactics of treatment is defined depending on a phase of a disease and expressiveness of clinical manifestations. In a chronic phase at an asymptomatic current and poorly expressed laboratory changes are limited to the all-strengthening actions. Sick chronic myeloid leukemia recommend to observe a work-rest schedule, to eat food, vitamin-rich etc. At increase in level of leukocytes use . After normalization of laboratory indicators and reduction of a spleen to patients with chronic myeloid leukemia appoint the supporting therapy or course treatment busulfany. The radiotheraphy is usually used at a leykotsitoza in a combination to a splenomegaliya. At decrease in level of leukocytes do a pause lasting not less than a month, and then pass to the supporting therapy busulfany.
In the progressing phase of chronic myeloid leukemia use of one himiopreparat or polychemotherapy is possible. Apply , or hloretilaminouratsit. As well as in a chronic phase, carry out intensive therapy to stabilization of laboratory indicators, in the subsequent pass to the supporting doses. Poliokhimioterapiya courses at chronic myeloid leukemia repeat 3-4 times a year. At blastny crises perform treatment by a hydroxycarbamide. At inefficiency of therapy use . At the expressed thrombocytopenia, anemias carry out transfusions of a trombokontsentrat and eritrotsitarny weight. At a chloromove appoint radiotheraphy.
Bone marrow transplantation is carried out in the first phase of chronic myeloid leukemia. Long remission manages to be reached at 70% of patients. In the presence of indications carry out a splenektomiya. The emergency splenektomiya is shown at a gap or threat of a rupture of a spleen, planned – at hemolytic crises, the "wandering" spleen, recidivous perisplenita and sharply expressed splenomegaliya which is followed by violation of functions of abdominal organs.
Forecast of chronic myeloid leukemia
The forecast at chronic myeloid leukemia depends on a set of factors, defining from which the moment of an initiation of treatment is (in a chronic phase, a phase of activation or in the period of blastny crisis). As adverse predictive symptoms of chronic myeloid leukemia consider significant increase in a liver and spleen (the liver acts from under edge of a costal arch on 6 and more than a cm, a spleen – on 15 and more than a cm), over 100x109/l, thrombocytopenia less than 150x109/l, more than 500Õ109/l, increase in level of blastny cages in peripheral blood to 1% and more, increase in total level of promiyelotsit and blastny cages in peripheral blood to 30% and more.
The probability of a failure at chronic myeloid leukemia increases in process of increase in quantity of signs. Infectious complications or heavy gemorragiya become a cause of death. Average life expectancy of patients with chronic myeloid leukemia makes 2,5 years, however at the timely beginning of therapy and the favorable course of a disease this indicator can increase to several decades.