Sharp myeloid leukosis
Sharp myeloid leukosis – the malignant disease of system of blood which is followed by uncontrollable reproduction of the changed leukocytes, decrease in quantity of erythrocytes, platelets and normal leukocytes. It is shown by the increased tendency to development of infections, fever, bystry fatigue, loss of weight, anemia, bleeding, formation of petekhiya and hematomas, bone and joints pains. Sometimes changes of skin and a swelling of gums come to light. The diagnosis is established on the basis of clinical symptoms and these laboratory researches. Treatment – chemotherapy, transplantation of marrow.
Sharp myeloid leukosis
The Sharp Myeloid Leukosis (SML) – malignant defeat of a myeloid sprout of blood. Uncontrollable proliferation of leucemic cages in marrow involves suppression of other sprouts of blood. As a result the quantity of normal cages in peripheral blood decreases, there are anemia and thrombocytopenia. The sharp myeloid leukosis is the most widespread sharp leukosis at adults. The probability of development of a disease sharply increases after 50 years. Average age of patients makes 63 years. Men and women of young and middle age suffer equally often. In the senior age group prevalence of males is observed. The forecast depends on a type of a sharp myeloid leukosis, five-year survival fluctuates from 15 to 70%. Treatment is performed by experts in the field of oncology and hematology.
Reasons of a sharp myeloid leukosis
Various chromosomal violations are an immediate cause of development of OML. Among the risk factors promoting development of such violations specify adverse heredity, ionizing radiation, contact with some toxic substances, reception of a number of medicines, smoking and diseases of blood. The probability of developing of a sharp myeloid leukosis increases at Blum's syndrome (low growth, a high pitched voice, characteristic features of the person and various skin manifestations, including hypo - or a hyperpegmentation, skin rash, an ichthyosis, ) and Fankoni's anemias (low growth, defects of pigmentation, neurologic disorders, anomalies of a skeleton, heart, kidneys and genitals).
The sharp myeloid leukosis rather often develops at patients with a Down syndrome. Also hereditary predisposition in the absence of genetic diseases is traced. At OML at close relatives the probability of developing of a disease increases by 5 times in comparison with average values on population. The highest level of correlation comes to light at monoovular twins. If the sharp myeloid leukosis is diagnosed for one twin, the risk at the second makes 25%. One of the major factors provoking OML are blood diseases. The chronic myeloid leukosis in 80% of cases is transformed to a sharp form of a disease. Besides, OML becomes frequent an outcome of a miyelodisplastichesky syndrome.
Ionizing radiation causes sharp myeloid leukoses at excess of a dose of 1 Gr. Incidence increases in proportion to a radiation dose. In practice stay in zones of atomic explosions and accidents on nuclear power plants, work with radiation sources without the corresponding protective equipment and the radiotheraphy applied at treatment of some oncological diseases matters. The marrow aplaziya as a result of mutations and defeat of stem cells is the reason of development of a sharp myeloid leukosis at contact with toxic substances. The negative impact of toluene and benzene is proved. Usually OML and other sharp leukoses are diagnosed 1-5 years later after contact with a mutagen.
Among the medicines capable to provoke sharp myeloid leukoses, experts call some medicines for chemotherapy, including DNK-topoizomerazy inhibitors II (, , doxorubicine and other antratsiklina) and alkiliruyushchy means (, , , hlorambutsit, , ). OML can also arise after reception of chloramphenicol, phenylbutazone and medicines of arsenic. The share of medicinal sharp myeloid leukoses makes 10-20% of total of cases. Smoking not only increases probability of development of OML, but also worsens the forecast. Average five-year survival and duration of full remissions at smokers is lower, than at non-smoking.
Classification of a sharp myeloid leukosis
Classification of a sharp myeloid leukosis according to WHO is very difficult and includes several tens of kinds of a disease divided into the following groups:
- OML with typical genetic changes.
- OML with the changes caused by a dysplasia.
- The secondary sharp myeloid leukoses which resulted from treatment of other diseases.
- Diseases with proliferation of a myeloid sprout at a Down syndrome.
- Myeloid sarcoma.
- Blastny plazmatsitoidny dendritnokletochny tumor.
- Other types of a sharp myeloid leukosis.
Treatment tactics, the forecast and duration of remissions at different types of OML can significantly differ.
Symptoms of a sharp myeloid leukosis
The clinical picture includes toxic, hemorrhagic, anemichesky syndromes and a syndrome of infectious complications. At early stages of display of a sharp myeloid leukosis are not specific. Temperature increase without signs of a catarrhal inflammation, weakness, fatigue, loss of weight and appetite is noted. At anemia dizzinesses, unconscious states and pallor of integuments join. At thrombocytopenia the raised bleeding and petekhialny hemorrhages are observed. Formation of hematomas at minor injuries is possible. At a leykopeniye there are infectious complications: frequent suppurations of wounds and scratches, persistent repeated inflammations of a nasopharynx etc.
Unlike a sharp limfoblastny leukosis at a sharp myeloid leukosis there are no expressed changes from peripheral lymph nodes. Lymph nodes are small, mobile, painless. Sometimes increase in lymph nodes in cervical and supraclavicular area comes to light. The liver and a spleen within norm or are slightly increased. Signs of defeat of the bone and articulate device are characteristic. Many patients with a sharp myeloid leukosis show complaints to pains of various degree of intensity in a backbone and the lower extremities. Restrictions of movements and change of gait are possible.
Among ekstramedullyarny displays of a sharp myeloid leukosis – gingivit and . The swelling of gums and increase in palatal almonds as a result of infiltration are in some cases observed by leucemic cages. At myeloid sarcoma (makes about 10% of total of cases of a sharp myeloid leukosis) on skin of patients there are greenish, is more rare – pink, gray, white or brown opukholevidny educations (hloroma, skin leykemida). Sometimes at damages of skin the paraneoplastic syndrome (a syndrome It is twisted), which is shown by an inflammation of integuments around leykemid is found.
In development of a sharp myeloid leukosis allocate five periods: initial or preclinical, heat, remission, recurrence and terminal. In an initial stage the sharp myeloid leukosis proceeds asymptomatically or is shown by nonspecific symptomatology. In the heat period the toxic syndrome becomes more expressed, anemichesky, hemorrhagic and infectious syndromes come to light. During remission of display of a sharp myeloid leukosis disappear. A recurrence proceeds similar to the heat period. The terminal period is followed by the progressing deterioration in a condition of the patient and comes to the end with a lethal outcome.
Diagnostics and treatment of a sharp myeloid leukosis
The crucial role in the course of diagnostics is played by laboratory analyses. Use the analysis of peripheral blood, a miyelogramma, microscopic and cytogenetic researches. For receiving a sample of fabrics carry out an aspiration biopsy of marrow (a sternalny puncture). In the analysis of peripheral blood of the patient with a sharp myeloid leukosis decrease in quantity of erythrocytes and platelets is found. The quantity of leukocytes can be as raised, and (more rare) lowered. In dabs blasta can come to light. The basis for diagnosis "the sharp myeloid leukosis" becomes detection more than 20% of blastny cages in blood or in marrow.
Basis of treatment of a sharp myeloid leukosis is the chemotherapy. Allocate two stages of treatment: induction and consolidation (postremissionny therapy). At a stage of induction carry out the medical actions directed to reduction of quantity of leucemic cages and achievement of a condition of remission. At a stage of consolidation eliminate the residual phenomena of a disease and prevent a recurrence. Medical tactics is defined depending on a type of a sharp myeloid leukosis, the general condition of the patient and some other factors.
The most popular scheme of induction treatment – "7+3", the tsitarabina providing continuous intravenous administration within 7 days in combination with simultaneous bystry periodic introduction of an antratsiklinovy antibiotic during the first 3 days. Along with this scheme in the course of treatment of a sharp myeloid leukosis other medical programs can be applied. In the presence of a serious somatic illness and high risk of development of infectious complications as a result of suppression of a myeloid sprout (usually – at patients of senile age) use less intensive palliative therapy.
The program of induction allows to achieve remission from 50-70% of patients with a sharp myeloid leukosis. However without further consolidation at most of patients there comes the recurrence therefore the second stage of treatment is considered as an obligatory part of therapy. The plan of consolidation treatment of a sharp myeloid leukosis is formed individually and includes 3-5 courses of chemotherapy. At high risk of a retsidivirovaniye and already developed recurrence transplantation of marrow is shown. Other methods of treatment of recurrent OML are in a stage of clinical tests so far.
Forecast of a sharp myeloid leukosis
The forecast will be defined by a kind of a sharp myeloid leukosis, age of the patient, existence or lack of a miyelodisplastichesky syndrome in the anamnesis. Average five-year survival at the OML different forms fluctuates from 15 to 70%, probability of development of a recurrence – from 33 to 78%. At elderly people the forecast is worse, than at young people what is explained by existence of the accompanying somatic diseases which are a contraindication for carrying out intensive chemotherapy. At a miyelodisplastichesky syndrome the forecast is worse, than at primary sharp myeloid leukosis and OML which arose against the background of pharmacotherapy concerning other oncological diseases.