With acute myeloblastic leukemia, malignant transformation and uncontrolled proliferation of abnormally differentiated, long-lived cell precursor cells causes the appearance of blast cells in circulating blood, replacement of normal bone marrow by malignant cells.

Code of the ICD-10

C92.0 acute myeloid leukemia

Symptoms and diagnostics of acute myeloblastic leukemia

Symptoms include fatigue, pallor, fever, infection, bleeding, easily formed subcutaneous hemorrhages; The symptoms of leukemic infiltration are available only in 5% of patients (often in the form of skin manifestations). To establish a diagnosis it is necessary to study the smear of peripheral blood and bone marrow. Treatment includes induction chemotherapy to achieve remission and post-remission therapy (with or without stem cell transplantation) in order to prevent recurrence.

The incidence of sharp myeloblastic leukemia increases with age, this is the most common leukemia in adults with the median age of the development of the disease, equal to 50 years. Acute myeloblastic leukemia can develop as a secondary oncological disease after chemotherapy or radiation therapy for different types Cancer.

Acute myeloblastic leukemia includes a series of subtypes that differ from each other in morphology, immunophenotype and cytochimia. On the basis of the predominant type of cells, 5 classes of acute myeloblastic leukemia are described: myeloid, myeloidomocytic, monocytic, erythitoid and megacocciratory.

Acute promoelocytar leukemia is a particularly important subtype and is 10-15% of all cases of acute myeloblastic leukemia. It meets at the most young group of patients (median age 31 years old) and mainly in a specific ethnic group (Latin American). This option often debuts blood coagulation disorders.

Treatment of acute myeloblastic leukemia

The purpose of the initial therapy of acute myeloblastic leukemia is to achieve remission, and, in contrast to acute lymphoblastic leukemia, with acute myelolomicosis, the answer is achieved when using fewer drugs. Remissance induction basic mode includes a long-term intravenous citationabine infusion or cytarabine in high doses within 5-7 days; During this time, double-biccine or idarubicin is administered intravenously. Some modes include 6-thioguanine, etoposide, vincristine and prednisone, but the effectiveness of the treatment of therapeutic schemes is unclear. Treatment usually leads to pronounced myelosuppression, infectious complications and bleeding; Before the restoration of the bone marrow usually passes a long time. During this period, careful preventive and supportive therapy is vital.

With acute proposal leukemia (OPL) and some other variants of acute myeloblastic leukemia, at the time of the diagnosis, disseminated intravascular coagulation (DVS), aggravated as a result of the release of leukemic cells of proagulatants. With acute proposal flow leukemia with translocation T (15; 17) The use of at-ra (transretinic acid) promotes the differentiation of blast cells and correction of disseminated intravascular coagulation within 2-5 days; In combination with a double-chip or omeubicin, this mode can induce remission in 80-90% of patients with long-term survival rate of 65-70%. Arsenic trioxide is also effective in acute promelocytic leukemia.

After reaching remission, the intensification phase is carried out by these or other drugs; Modes using cytarabina in high doses can increase the duration of remission, especially in patients under 60 years. Prevention of lesion central nervous system It is usually not carried out, since with sufficient systemic therapy, the defeat of the central nervous system is a rare complication. In patients who received intensive treatment, there was no advantage of supporting therapy, but in other situations it can be useful. Extramedullary defeat as an isolated recurrence is rare.

MKB 10 or international Classification Of all diseases of the 10 convocation, there are almost all short designations of famous pathologies, including oncological ones. Leukemia briefly on the ICD 10 has two accurate encodings:

• C91. - lymphoid form.
• C92. - myeloid form or myelolomicosis.

But also need to take into account the nature of the disease. For the designation, use a subgroup that is written after the point.

Lympholoikosis

Mieloloikosis

Includes granulocyte and myelogenic.

The reasons

Recall that the exact cause is due to the development of blood cancer is not known. That is why doctors, it is so difficult to deal with this ailment and prevent it. But there are a number of factors that can increase the chance of the oncology of red liquid.

• Increased radiation
• Ecology.
• Poor food.
• Obesity.
• Excessive use of medicines.
• Excess weight.
• Smoking, alcohol.
• Harmful work associated with pesticides and chemical reagents that can affect the hematopoietic function.

Symptoms and anomalies

• Anemia arises as a result of oppression of erythrocytes due to which oxygen to healthy cells does not reach full.
• Strong and frequent headaches. Begins with 3 stages when intoxication arises due to malignant tumor. There may also be the result of running anemia.
• Constant cold and infectious and viral diseases With a long period. It happens when healthy leukocytes are replaced by atypical. They do not fulfill their function and the body becomes less protected.
• Pain in the joints and breaking.
• Weakness, fatigue, drowsiness.
• Systematic subfebrile temperature for no reason.
• Change odor, tastes.
• Weight loss and appetite.
• Long bleeding with a decrease in blood platelets.
• Soreness inflammation of lymph nodes throughout the body.

Diagnostics

The exact diagnosis can be delivered, only after a thorough examination and passing a certain list of analyzes. Most often people catch on abnormal indicators with biochemical and general Analysis blood.

For more accurate diagnosis, the bone marrow puncture is made from pelvic bone. Later, the cells are sent to the biopsy. Also, an oncologist is also conducting a complete inspection of the body: MRI, ultrasound, CT, X-ray, to identify metastases.

Treatment, therapy and forecast

The main type of treatment is chemotherapy, when chemical poisons are introduced into the blood, which are aimed at the destruction of abnormal blood cells. The danger and the little efficiency of this type of treatment is that healthy blood cells, which are also destroyed and so little.

When identifying the primary hearth, the doctor may appoint chemistry to completely destroy the bone marrow in this area. After the procedure, irradiation can also be carried out to destroy cancer cells. In the process there is a stem cell transplant from the donor.

Ministry of Health of the Irkutsk Region

ORDER

On approval of an outpatient standard for medical care at sublececemic myelose

In order to improve the quality of rendering medical care The population of the Irkutsk region, in accordance with the principles of the legislation of the Russian Federation on the protection of the health of citizens of the Russian Federation, guided by clause 9 of the Regulations on the Ministry of Health of the Irkutsk Region, approved by the Government of the Irkutsk Region of October 7, 2008 N 13-PP, I order:

1. To approve the attached outpatient standard for the provision of medical care at sublececemic myelose.

2. This Order is subject to official publication in the Regional newspaper.

3. Control of the execution of the order to entrust to the head of the Office of the Organization of Medical Aid of the Ministry of Health of the Irkutsk Region Gavrilov L.L.

Minister
G.M.Gaidarov

Application. Ambulatory standard for medical care at sublececemic myelose

Annex to order

ministry of Health

Irkutsk region

1. Patient model

Nonological shape: sublecemic myelosis.

Code of ICD-10: D47.1

Phase: any.

Stage: any.

Complications: regardless of complications.

Terms of Rendering: Ambulatory and Polyclinic Help.

1.1. DIAGNOSTICS

2.1. Treatment at the rate of 365 days

Leukemia

Acute leukemia.

Chronic lympholoicosis.

Chronic myelolomicosis.

True polycythemia.

Acute leukemia

Definition.

Acute leukemia is a myeloproliferative tumor, the substrate of which are blades, deprived of the ability to differentiate into mature blood cells.

ICB10:C91.0 - acute lymphoblastic leukemia.

C92.0 - acute myeloid leukemia.

C93.0 - acute monocystary leukemia.

Etiology.

Latent viral infection, predisposing heredity, the effects of ionizing radiation are able to cause somatic mutations in the hematopoietic fabric. Among mutant polypotent cells close to the stem cell can be formed a clone insensitive to immunoregulatory influences. From the mutant clone is formed intensively proliferating and metastatic beyond the bone marrow tumor consisting of blasts of one species. A distinctive feature Tumor blasts are the inability to further differentiate into mature blood cells.

Pathogenesis.

The most important link of the pathogenesis of acute leukemia is a competitive metabolic suppression by anomalous blasts of the functional activity of normal hematopoietic fabric and displacing it from the bone marrow. As a result, aplastic anemia, agranulocytosis, thrombocytopenia with characteristic hemorrhagic syndrome, severe infectious complications due to deep violations in all immunity links, deep dystrophic shifts in the tissues of internal organs are arising.

According to the Fab classification (a cooperative group of hematologists of France, America and Britain, 1990) allocate:

Acute lymphoblastic (lymphoid) leukemia.

Acute Nelimfoblastic (myeloid) leukemia.

Acute lymphoblastic leukemia are divided into 3 types:

L1 - acute microlimfoblastic type. Antigenic markers of blasts correspond to zero ("neither t, n n") or thymus-dependent (T) lines of lymphopower. It is found mainly in children.

L2 - acute lymphoblastic. Its with a substrate - typical lymphoblasts whose antigenic markers are the same as with L1 type of acute leukemia. More often occurs in adults.

L3 is an acute macrolymphocytic and prolimphocytic leukemia. Blasts have antigenic markers of in-lymphocytes, morphologically similar to the cells of Berkitt lymphoma. This type is rare. It has a very bad forecast.

Acute Nelimfoblastic (myeloid) leukemias are divided into 6 types:

M0 - acute undifferentiated leukemia.

M1 - acute myeloblastic leukemia without cell aging.

M2 - acute myeloblastic leukemia with signs of cell aging.

M3 - acute promelocytar leukemia.

M4 - acute myelonoblastic leukemia.

M5 - acute monoblastic leukemia.

M6 - acute erythroeelosis.

Clinical picture.

In the clinical course of acute leukemia, the following stages are distinguished:

The initial period (primary acting stage).

In most cases, it begins acutely, often in the form of "influenza". Suddenly the body temperature rises, chills appear, throat pain, arthralgia, sharply pronounced general weakness. Less frequently, the disease can first manifest itself with thrombocytopenic purple, recurrent nose, uterine, gastric bleeding. Sometimes OL starts with a gradual deterioration of the patient's condition, the emergence of non-pronounced arthralgies, pain in the bones, bleeding. In isolated cases, an asymptomatic principle is possible.

In many patients in the initial period, an increase in peripheral lymph nodes, moderate splenomegaly, is revealed.

Stage of deployed clinical hematological manifestations (first attack).

Characterized sharp deterioration The general condition of the patients. Typical complaints about pronounced general weakness, high fever, pain in the bones, in the left hypochondrium in the field of spleen, bleeding. At this stage, clinical syndromes are formed, typical for Ol:

Hyperplastic (infiltrative) syndrome.

An increase in lymph nodes and spleen is one of the most typical manifestations of the dissemination of the leukemic tumor. Leukemic infiltration often causes podcapsulable hemorrhages, heart attacks, sprayer breaks.

The liver and kidneys are also increased due to leukemic infiltration. Leukemian filtrates in the lungs, pleura, mediastinal lymph nodes are manifested by the symptoms of pneumonia, exudative pleurrites.

Leukemic infiltration of gums with their swelling, hyperemia, ulceratives. Conventional phenomenon for acute monocytic leukemia.

Localized tumor masses (leukemides) in the skin, eyeballs, in other places occur during non-cellular (myeloid) forms of leukemia in the later stages of the disease. In some myeloblast leukemia, leukemides may have a greenish color ("chlorom") due to the presence of myeloperoxidase tumors in blast cells.

Anemic syndrome.

Leukemia infiltration and metabolic oppression of normal sprouts of bone marrowing leads to the emergence of aplastic anemia. Usually anemia is normal. In acute erythroeelosis, it may have a hyperchromic megaloblastoid character with a moderately pronounced hemolytic component. With pronounced splenomegaly, hemolytic anemia may occur.

Hemorrhagic syndrome.

Due to thrombocytopenia, DVS syndrome. It is manifested by subcutaneous hemorrhages (thrombocytopenic purpura), bleeding of gums, nose, uterine bleeding. Gastrointestinal, pulmonary bleeding, macrohematuria are possible. Along with hemorrhages, thrombophlebitis, thromboembolism, other hypercoagulative disorders caused by the Food Syndrome occur. This is one of the characteristic manifestations of acute promelocytic and myelonoblastic leukemia.

Immunodeficiency syndrome.

The formation of the immunodeficiency state is due to the displacement by leukemic blasts of normal clones of immunocompetent cells from the bone marrow. Clinically manifested by fever, often hectic type. Foci of chronic infection of different localization appear. The occurrence of ulcer necrotic angina, peritonzillary abscesses, necrotic gingivitis, stomatitis, pyodermia, paragreught abscesses, pneumonia, pyelonephritis is characteristic. Generalization of infection with the development of sepsis, multiple abscesses in the liver, kidney, hemolytic jaundice, the DVS syndrome is often the cause of the patient's death.

Neurolemia syndrome.

It is characterized by the metastatic distribution of foci of blast proliferation in brain shells, the substance of the brain, structure spinal cord, nervous trunks. Manifests by meningial symptoms - headache, nausea, vomiting, violations of view, the rigidity of the occipital muscles. The formation of large tumor-like leukemic infiltrates in the brain is accompanied by focal symptomatics, paralymps of the cranial brain nerves.

Remissance achieved as a result of treatment.

Under the influence of the treatment conducted, extinction (incomplete remission) occurs or even complete disappearance (complete remission) of all clinical manifestations of the disease.

Recurney (second and subsequent attacks).

As a result of the ongoing mutations, there is a clone of tumor blasts capable of "evclusing" from the effects of cytostatic drugs used for supporting treatment. There is an aggravation of the disease with the return of all syndromes typical for stages of deployed clinical and hematological manifestations of OL.

Under the influence of anti-relapse therapy, remission can again be achieved. The optimal treatment tactics can lead to recovery. If you are insensitive to the treatment of ol, it goes into the terminal stage.

Recovery.

The patient is considered recovered if complete clinical and hematological remission persists more than 5 years.

Terminal stage.

It is characterized by deficiency or complete absence of therapeutic control over the growth and metastasis of the leukemic clone of the tumor. As a result of diffuse infiltration of the bone marrow, the internal organs leukemic blasts is totally suppressed by the system of normal blood formation, infectious immunity disappears, there are deep violations in the hemostasis system. Death comes from disseminated infectious lesions, non-coming bleeding, severe intoxication.

Clinical features of the morphological types of acute leukemia.

Acute undifferentiated leukemia (M0). It is rare. Very quickly progresses with the aggravation of severe aplastic anemia, severe hemorrhagic syndrome. Remissions are rarely achieved. The average life expectancy is less than 1 year.

Acute myeloblastic leukemia (M1-M2). The most common variant of acute nonlimfoblastic leukemia. Adults are more often sick. It is difficult, persistently progressive with pronounced anemic, hemorrhagic, immunosuppressive syndromes. Non-necrotic lesions of the skin, mucous membranes are characteristic. To achieve remission is possible in 60-80% of patients. The average life expectancy is about 1 year.

Acute proposal leukemia (m3). One of the most malignant options. It is characterized by pronounced hemorrhagic syndrome, which most often leads to a patient to death. Stormy hemorrhagic manifestations are associated with DVS syndrome, the cause of which is to increase the thromboplastine activity of leukelocytes. On their surface and in the cytoplasm contains 10-15 times more thromboplastin than normal cells. Timely treatment allows you to achieve remission almost every second patient. The average life expectation reaches 2 years.

Acute myelonoblastic leukemia (M4). The clinical symptoms of this form of the disease is close to acute myeloblastic leukemia. Differences are more inclined to necrosis. More often arises in DVS syndrome. Each tenth patient has neuroleycosis. The disease quickly progresses. Often severe infectious complications arise. The average life expectancy and frequency of persistent remission are two times less than in acute myeloblastic leukemia.

Acute monoblastic leukemia (M5). Rare shape. Clinical manifestations differ little from myelonoblastic leukemia. It has a greater inclination to rapid and stubborn progression. Therefore, the average life expectancy of patients with this form of leukemia is even less - about 9 months.

Acute erythroeelosis (M6). Rare shapes. A distinctive feature of this form is stubborn, deep anemia. Hyperchrome anemia with expressed hemolysis of nonresco. In leukemic erythroblasts, megaloblastoid deviations are detected. Most cases of acute erythromelosis resistant to therapy. The life expectancy of patients rarely exceeds 7 months.

Acute lymphoblastic leukemia (L1, L2, L3). This form is characterized by a moderately progressive course. Accompanied by an increase in peripheral lymph nodes, spleen, liver. Hemorrhagic syndrome, ulcer-necrotic complications are rare. Lifespan with acute lymphoblastic leukemia from 1.5 to 3 years.

Information:Lakeeucoses -Temmin, combining numerous tumors of the hematopoietic system arising from blood-forming cells and affecting the bone marrow. The separation of leukemias into two main groups - common and chronic-is determined by the structure of tumor cells: leukemia, the cell substrate is presented to the acute, and to chronic-delicates, in which the bulk of tumor cells is differentiated and consists mainly of mature elements. The duration of the disease does not determine the assignment of one or another leukemia to the group of sharp or chronic. Etiology, pathogenesis. The reason for the occurrence of acute leukemia and chronic myelolecosis of a person may be a violation of the composition and structure of the chromosomal apparatus, hereditably determined or acquired under the influence of certain mutagenic factors. One of them is ionizing radiation. The cause of the development of leukemia is also the effect of chemical mutagens. The increase in acute leukemia among individuals subjected to benzene, as well as among patients who received cytostatic immunosuppressants (imuran, cyclophosphane, leucaran, sarcolizin, Mustargen, etc.); The frequency of sharp leukemia among this contingent of patients rises hundreds of times. The facts of the occurrence of acute myeloblastic leukemia, acute erythromelosis are known against the background of long-term chemotherapy of chronic lympholecosis, macrooglobulinemia of Valdenstrem, myeloma, lymphogranulosis and other tumors. The role of hereditary defects in myeloid and lymphatic tissue predisposing glukes. The observations of the dominant and recessive inheritance of chronic lympholecosis are described, the low incidence of these leukemia in some ethnic groups is noted and increased in others. More often in these cases, the leukemia itself is inherited, but the increased variability is the unstability of chromosomes, predisposing the helicate myeloid or lymphatic cells to leukemic transformation. The use of chromosomal analysis made it possible to establish that with any leukemia, the clone of tumor leukemous-descendant cells of one initially mutated cell occurs. The instability of the genotype of malignant cells during leukemates causes the appearance in the original tumor clone of new clones, among which in the course of the body's life activity, as well as under the influence of medical agents "Selected" the most autonomous clones. This phenomenon explains the prishability of the flow of leukemias, their departure from under the control of cytostatics. The leukemia is sharp. According to morphological (mainly cytoochemical) criteria, the following main forms of acute leukemia are distinguished: lymphoblastic, myeloblastic, monoblacitic, megacaroblastic, erypromelosis, plaszoballastic, undifferentiated, low-rise acute leukemia. For all acute leukemia, the increasing "unpretentious" weakness, ailments, sometimes shortness of breath, dizziness caused by anemia. Increased body temperature, intoxication - frequent symptoms of non-cellular leukemia. The increase in lymph nodes, liver and spleen in the expanded stage is not occurring at all acute leukemia, but can develop independently of the form of acute leukemia in the terminal stage. Hemorrhagic syndrome is often due to blood-bootopenia: bleeding of mucous membranes, phetechial rash on the skin, especially the legs. Lungs, myocardium and other fabrics and organs may appear leukemic blast infiltrates. Diagnosis of sharp leukemia is based on data cytological research blood and bone marrow discovering a high percentage of blast cells. In the early stages, there are usually no of them, but the cytopenia is expressed. Therefore, when cytopenia, even relating to one sprout, a bone marrow puncture is necessary, which can be made outpatient. IN bone marrow It is noted high (tens of percent) The content of blasts with all acute leukemia, with the exception of acute low-rise leukemia, in which for many months in the blood and bone marrow the percentage of blast cells may be less than 15-20, and in the bone marrow in this form, as a rule , the percentage of blasts is less than in the blood. The form of acute leukemia is installed using histochemical methods. The most frequent forms of acute leukemia in adults are myeloblastic and myelonoblastic leukemia. At the beginning of the disease, with these forms, the liver and spleen usually normal sizes, the lymph nodes Not increased, together with those, deep granulocyptopenia, anemia, thrombocytopenia. Incixation is often expressed, the body temperature is increased. Grunt cells have structural kernels with a delicate chromatin network, often several small nucleol; The cytoplasm of blast cells contains azurophilic graininess or auer taurus, which give a positive response to peroxidase and lipids. With myhellonoblastic leukemia in the cytoplasm, not only these substances, but also alpha-naphthylterase, characteristic of the elements of the monocytic series, are detected; Alpha-naphthylterase is suppressed by sodium fluoride. Acute lymphoblastic leukemia is more common in children. As a rule, from the very beginning proceeds with lymphoenopathy, an increase in the spleen, food. In the blood, only moderate normochromic anemia, leukopenia, but in the bone marrow, can be observed, but in the bone marrow - total blastosis. Blast cells have a rounded core with a gentle chromatin network and 1-2 nucleolas, a mesmer-free narrow cytoplasm. With a chic reaction in a cytoplasm, glycogen's boulders are detected, focused in the form of a necklace around the kernel. Acute Promipocytic leukemia occurs quite rarely; Until recently, it was characterized by the speed of course. It has pronounced intoxication, bleeding and hypofibrinegenemia due to the engineering of the engine. Lymph nodes, liver and spleen are usually not increased. In the hemogram of anemia, severe thrombocytopenia, in the bone marrow a large percentage of atypical blasts. The power cells of various sizes and shapes have a cytoplasm, thickly filled in some cells with large purple-brown graininess, located on the core, in other-shallow abundant azurophilic graininess; Frequently, Taurus Auer. The graininess contains acidic sulfated mucopolysaccharides. The cores of these leukemian cells in the blood often have a two-grate form, even more often their form is difficult to distinguish between abundance of grainability in the cytoplasm. The immediate cause of the death of the patient most often has hemorrhage into the brain. Acute monoblastic leukemia is relatively rare. A typical beginning of this form differs little from myeloblastic, but intoxication and an increase in body temperature to febrile digits are more pronounced. A frequency symptom is the gumsion mucosa hyperplasia due to leukemic proliferies in them. In the blood, the granulocytic sprout can be relatively preserved at first, along with Blastny, many mature, to a greater or lesser degree of ugly monocytes, are found. Grunt cells have a bean-shaped structural core with several nucleolas and a grayish-blue cytoplasm sometimes with a scanty azurophilic graininess. Cytochemically revealed a positive reaction to alpha-naphthylterase, suppressed by sodium fluoride, weakly-bedding reaction to peroxidase and lipids. In the serum and the urine of these patients high level of lysozyme. Acute plasmoblastic leukemia is characterized by the appearance of plasmoblasts and plasmocytes in the bone marrow and plasmocytes with features of cells; In addition, there are many non-differentiable blasts. The characteristic cytochemical signs of this form of acute leukemia are unknown; Its feature is the detection of paraprotein in serum. Extramedullary leukemic foci is often expressed, an increase in lymph nodes, liver, spleen, leukemides in the skin, testicles. Acute megakaryoblastic leukemia is very rare. It is characterized by the presence in the bone marrow and blood of megakaryoblasts (cells with a blast, but hyperchromic core, narrow cytoplasm with filamentous growths), as well as undifferentiated blasts. Often in the blood and bone marrow there are ugly megakaryocytes and fragments of their nuclei. Characterized thrombocytosis (more than 1000- Lo (in the fourth degree) of μl). Acute erythroeelosis occurs relatively rarely. The disease is characterized by the hyperplasia of the cells of the red row without signs of sharp hemolysis. Clinical symptoms: progression of normo or hyperchromic anemia without reticulocytosis (usually up to 2%), non-timbble due to the decay of erythrocarocytes, increasing leukopenia and thrombocytopenia. The bone marrow was increased the content of red row cells with the presence of multi-core erythroblasts and undifferentiated power cells. In contrast to other forms of acute leukemia, the tumor cells of the red row are often differentiated to the stage of oxificial normocitis or to the red blood cell. Acute erythroeelosis is often transformed into acute myeloblastic. Neurolekemia is one of the frequent complications of acute leukemia, less often chronic myelolecosis. Neurolecemia is a leukemic lesion (infiltration) of the nervous system. Especially often this complication occurs in acute lymphoblastic leukemia of children, less often - with other forms of acute leukemia. The occurrence of neurolekemia is due to the metastasis of leukemic cells into the shell of the head and spinal cord or in the brain substance (prognostically it is a heavier type of tumor growth). The neurolemia clinic is consisted of meningeal and hypertensional syndromes. Noted persistent headache, re-vomiting, lethargy, irritability, swelling of disks spectator nerves, nistagm, squint and other signs of defeat of cranial nerves and meningkeal signs. IN spinal fluid High worse cytosis. The detection of high cytosis and blast cells in the spinal fluid is an earlier sign of nailarecemia than the described clinical picture. In intracerebral metastases - the pattern of brain tumor without cytosis. Treatment. In acute leukemia, urgent hospitalization is shown. In some cases, with an accurate diagnosis, cytostatic treatment is possible in outpatient conditions. Apply pathogenetic treatment To achieve remission with the help of combined administration of cytostatics in order to eliminate all explicit and alleged leukemic foci, the pronounced hematopopian depression is possible. The remission in acute leukemia is called a condition in which the level of platelets above 10 -104 in 1 μl, leukocytes-needed-only μl, in the bone marrow of blasts less than 5%, and the lymphoid cells are less than 30%, there are no extra-visual leukemic proliferates. In acute lymphoblastic leukemia of children, the mandatory criterion of completeness of remission is the normal composition of the spinal fluid. In children suffering from acute lymphoblastic leukemia, the combination of vincristine appointed in a dose of 1.4 mg / m2 (no more than 2 mg) is most effective (no more than 2 mg) 1 time per week in / in, and prednisone inside every day at a dose of 40 mg / m2. With this therapy, remission is achieved about 95% of children in 4-6 weeks. Already during the period of reaching remission, neurolemia prevention is beginning: the first cerebral puncture should be done the next day after the diagnosis of acute lymphoblastic leukemia is established and at the same time introduce intrablum methotrexate (AMETOPTERIN) at a dose of 12.5 mg / m2. Spinal punctures with the introduction of methotrexate in the specified dose repeated every 2 weeks before receiving remission. Immediately upon reaching remissions, a special preventive course is carried out, which includes the irradiation of the head at a dose of 2400 rad from double-told fields with the capture of I and II cervical vertebrae, but with eye protection, mouth, the entire facial skull area, and the simultaneous 5-fold (for 3 weeks) Intratubal administration of methotrexate in the same dose (12.5 mg / m2). When diagnosing neurolekemia during the lumbal puncture, the prophylactic irradiation of the head is canceled, carried out therapy of neurolemia with the help of the intralumless administration of two cytostatic drugs: methotrexate at a dose of 10 mg / m2 (maximum 10 mg) and cytosar (the initial dose of 5 mg / m2 is gradually increased to 30 mg / m2). During the remission of acute lymphoblastic leukemia in children, three cytostatic cytostatic therapy with three cytostatics - 6-mercaptopurine (50 mg / m2 per day) daily, cyclophosphane (200 mg / m21 times a week), methotrexate (20 mg / m21 once a week); Treatment continues 3.5-5 years. With acute lymphoblastic leukemia in adults and children with adverse initial testimony (late and interrupted treatment prior to admission to program therapy, age over 10-12 years old, the initial level of leukocytes is more than 20,000 in 1 μl) in the first week of remission received under the program comprising vincristine, prednisone and regulation, prescribe one of the cytostatic combinations: SAR, or CHOP, or POMP. The combination of SAR consists of cyclophosphane and cytosar administered from 1-to 4th day of the courses of 50 mg / m2 3 times a day with a syringe; Wincristine introduced at a dose of 1.4 mg / m2 V / in in the 1st day, and prednisolone given daily from the 1st to the 4th day at a dose of 100 mg / m2. The combination of CHOP consists of a cyclophosphane introduced in / in a dose of 750 mg / m2 in 1-day course, adriamycin - 50 mg / m2 V / in 1-day, vincristine - 1.4 mg / m2 (maximum 2 mg ) In the 1st day in / in and prednisone, given daily from 1st to the 5th day of the course at a dose of 100 mg / m2 per day. The POMP combination is designed for a 5-day course, comprising 6-mercaptopurine (purinetol) of 300-500 mg / m2 per day inward from 1st to 5th day, vincristine - 1,4 mg / m2 V / in 1st Day, methotrexate - 7.5 mg / m2 V / in daily from the 1st to the 5th day and prednisone, appointed inside a daily 200 mg / m2 per day. One of these courses are carried out at the beginning of remission to secure it (consolidation). Then (after exiting the cytopenia - the rise of the leukocyte level up to 3000 cells in 1 mm3) begin to therapy for maintaining remission; In acute lymphoblastic leukemia, it is carried out continuously the same three preparations (6-mercaptopurine, methotrexate and cyclophosphane), as in children 2-10 years old, but every one and a half months instead of this therapy appointed inside in tablets or, as cyclophosphamide, in powder, Conduct alternately. SAAR, CHOP or POMP (for all the time of maintenance therapy, i.e., on 5 Pet, choose any two of these three courses for a given patient). Regardless of age, patients with acute lymphoblastic leukemia are carried out prevention of neurolemia with two cytostatic drugs: methotrexate (10 mg / m2, maximum 10 mg) and cytosair (in an increasing dose from 5 to 30 mg - only 5 intralumless administrations) or head irradiation (in the sum2 of the Cream 15 sessions) and methotrexate administered intraborm 5 times simultaneously with irradiation at a dose of 12.5 mg / m2. With acute non-cellular leukes, the main drugs used to achieve remission are cytoosar and regulation (or adriamycin). They can be appointed in combination "7 + Z": the cytoosar is introduced 7 days continuously in daily dose 200 mg / m2 or 2 times a day every 12 h to 200 mg / m2 for 2 hours in / in; RUBUNIES is introduced in a syringe in a dose of 45 mg / m2 (30 mg / m2 to persons over 60 years old) in the 1st, 2nd and 3rd days of the course. To cytoosor and the regulation, 6-mercaptopurine, prescribed every 12 h in a dose of 50 mg / m2, can be added, while the dose of cytosar is reduced to 100 mg / m2 administered every 12h. Citoosar is introduced 8 days, 6-mercaptopurine - from the 3rd to 9th day. When remission reaches the fixing course - consolidation - may be the same as leaving for remission. To maintain remissions, or the same combination of cytosar and the regulation (course "7 + 3"), appointed every month with an interval of 2.5 or 3 weeks, or a 5-day administration of cytoosor p / k at 100 mg / m2 every 12 hours in Combination (on the first day of the course) with one of these cytostatics as cyclophosphane (750 mg / m2) or a regulation (45 mg / m2) or vincristine (1.4 mg / m2 in 1st day) and prednisone (40 mg / M2 from the 1st to the 5th day) or methotrexate (30 mg / m2). Supporting therapy continues 5 years, as in acute lymphoblastic leukemia. All patients are carried out by neurolemia prevention. The first lumbar puncture with the introduction of methotrexate at a dose of 12.5 mg / m2 (maximum 15 mg) is made with all forms of acute leukemia in all age groups in the first days after the diagnosis of acute leukemia. In adults, the main course of neurolemia prevention is carried out after remission; In children with acute lymphoblastic leukemia, in the period of induction of remission every 2 weeks, methotrexate in a dose of 12.5 mg / m2 (maximum of 15 mg) are re-introduced. In the case of reactions before administration, prednisolone V / V at a dose of 120 mg is prescribed. Chronic leukemia. More often there are lympholoicosis, myelolomicosis, myeloma disease, eritremium, less frequent-chronic sublecemic myelosis (osteomyelosclerosis, myelofibrosis), chronic monocystary leukemia, macroglobulinemia of Valdenstrem. In chronic myelolomicosis, the tumor process is affected both granulocyte and thrombocytar and erythrocytarian sprouts of the bone marrow. Podonachal of the tumor-cell predecessor myelopoose. The process can spread to the liver, spleen, and in the terminal stage there may be any tissue. IN clinical flow Chronic myelolecosis is distinguished by the deployed and terminal stage. At the beginning of the expanded stage, there are no complaints in the patient, the spleen was not increased or slightly increased, the composition of peripheral blood is changed. In this stage, the diagnosis can be established by analyzing the "unmotivated" nature of neutrophilic leukocytosis with a shift in the formula to myelocytes and promoelocytes, the detection of a significantly increased ratio of leukocytes / erythrocytes in the bone marrow and the "Philadelphia" chromosome in blood granulocytes and bone marrow cells. In the bone marrow treanate already during this period, as a rule, there is almost a complete outstanding of fat myeloid tissue. The deployed stage can continue on average 4 years. With proper therapy, the patient's condition remains satisfactory, they maintain ability to work, lead a normal lifestyle with outpatient observation and treatment. In the terminal stage, the course of chronic myelolecosis acquires the features of malignancy: high fever, fast progressive exhaustion, bone pain, sharp weakness, rapid increase in spleen, liver, sometimes an increase in lymph nodes. For this stage, the appearance and rapid increase in the signs of suppressing normal hematopopia - anemia, thrombocytopenia, complicating hemorrhagic syndrome, granulocyptopenia, complicating infection, necrosis of mucous membranes. The most important hematological sign of the terminal stage of chronic myelolecosis is Blastic Crisis - an increase in the content of blast cells in the bone marrow and blood (first more often myeloblasts, then non-differentiable blasts). Kariology in the terminal stage in more than 80% of cases, the appearance of aneupo -ide clones of cellular cells containing an abnormal number of chromosomes is determined. The duration of the patient's life in this stage does not exceed 6-12 months. Treatment of chronic myelolecosis is carried out from the moment of establishing a diagnosis. In the deployed stage, the treatment of myelosan in a dose of 2-4 mg / day (at the level of leukocytes more than 100,000 in 1 mm3 is prescribed to 6 mg / day). Treatment is carried out outpatient. With the inefficiency of myelosan, myelobromol is prescribed (with significant splenomegaly, the spleen is irradiated). When the process transition to the terminal stage, combinations of cytostatic drugs are used, usually used for the treatment of acute leukemia: vincristine and prednisone, vamp, cytoosar and RUBU). At the beginning of the terminal stage, myelobromol is often effective. Chronic lympholoicosis is a benign tumor of the immunocompetent system; The base of the tumor is morphologically mature lymphocytes. The beginning of the disease is often not possible to determine: among the complete health and the absence of a patient, some unpleasant subjective sensations in the blood are found small, but gradually increasing lymphocytosis. In the early stages, the number of leukocytes may be normal. Characteristic sign Diseases - an increase in lymph nodes. Sometimes their increases are discovered simultaneously with changes in blood, sometimes later. An increase in the spleen is a symptom; less often the liver is increasing. In the blood, along with an increase in the number of lymphocytes, the presence of single prolimphocytes and sometimes rare lymphoblasts can often be noted characteristic of chronic lympholecosis, the so-called shadows of the gumpracht - destroyed when preparing a core of lymphocytes, in which nucleoles can be seen among the side of chromatin. In the expanded stage of the disease, the content of neutrophils, platelets and erythrocytes may remain on normal level. In the bone marrow, a high percentage of lymphocytes is found in chronic lymphocyte. The development of the disease is often accompanied by a decrease. general level Gamma Globulinov. The oppression of humoral immunity is manifested by frequent infectious complications, especially pneumonia. Another incredible complication - cytopenia, more often anemia and thrombocytopenia. This complication may be due to the appearance of autoantibodes against erythrocytes and platelets or against erythrocariocytes and megacariocytes. But this is not the only cytopenia mechanism in chronic lympholoicosis; The inhibitory effect of lymphocytes (in particular, T-lymphocytes) on erythropoese or thrombocytopower precursor cells is possible. The terminal stage of chronic lympholecosis, manifested by a sarcoma rising or blast crisis, is observed infrequently, especially rare prosthest crisis. The development of lymphosarcoma in some cases may be accompanied by a change in blood lymphocytosis with neutropylise. Hairy-cell leukemia is a special form of chronic lympholecosis, in which the lymphocytes have a homogeneous core, resembling the nucleus of blasts, the vigilant growths of the cytoplasm. The cytoplasm of these cells contains many acid phosphatase, resistant to tartaric acid. Clinical picture It is characterized by an increase in the spleen, a minor increase in peripheral lymph nodes and pronounced cytopenia. In 75% of cases of hairy cell leukemia, which flows with an increase in the spleen, splenectomy is effective. If cytopenia is not associated with an increase in the spleen or there are any other organ changes or lymphadenopathy, the therapy of choice is the use of "alpha interferon (3,000,000-9,000,000 units per day for many months, taking into account the positive dynamics of blood indicators, changes in Affected fabrics). A separate form is a chronic lympholoicosis with leather damage - the shape of Cesari. The process begins often from lesions of the skin, skin itching, the appearance of local lymphatic infiltrates under the epidermis, which can then become total. The lymphocytosis and the percentage of ugly lymphocytes in the blood are gradted. This is usually large cells with rugged looped kernel contours, but cells can be small with a bean-like core. The belonging of these lymphocytes to T cells has been proven. Lymphadenopathy can be mixed nature: some lymph nodes are increased reactive due to infection in the skin, others - in connection With their leukemic infiltration. The spleen may uve Torture in the disease. In the treatment of the sisari form, the effect of the long-term use of small doses of chlorobutin (2-4 mg / day daily for several months is controlled by blood tests, primarily platelet levels - 1 time in 2-3 weeks), which removes skin itch, reduces leaky skin infiltration. Treatment of chronic lympholecosis, manifested by increasing leukocytosis, moderate lymphadenopathy, begin with the use of chlorobutin. With significant sizes of lymphatic nodes use cyclophosphane. Steroid therapy is prescribed with autoimmune complications, hemorrhagic syndrome, as well as the inefficiency of individual cytostatics (in the latter case, chlorubutin or cyclophosphane with prednisone) are sometimes combined. Long use Steroids with chronic lympholoicosis is contraindicated. With a significant density of peripheral lymph nodes, involvement in the process of lymph nodes abdominal cavity A combination of drugs such as vamp or combination of cyclophosphane, vincristine or vinblastine and prednisone (litter or CVP) is used successfully. They irradiate the spleen, lymph nodes, skin. One of the methods of treatment of autoimmune cytopenia in chronic lymphole is splenectomy. Of particular importance is the treatment of infectious complications. Recently, leukocytoisheresis for the treatment of lympholecosis with high leukocytosis and cytopenia began to apply. Sick chronic lympholes for many years retain good health and ability to work. Chronic monoritarian leukemia refers to the rare forms of leukemia, is characterized by high monocytosis in peripheral blood (20-40%) with a normal or somewhat elevated number of leukocytes. Along with mature monocytes in the blood there are single industrocytes. In the bone marrow, the percentage of monocytes is raised slightly, but in Trepanate there is a hyperslasia of bone marrow tissue with diffuse growth of monocytic elements. In the blood and urine high content of lysozyme. 50% of patients are palpated by a spleen. A long safe flow of chronic monocytic leukemia can change the terminal stage with the same features as the terminal stages of chronic myelolecosis. In the expanded stage, the process does not require special treatment, only with deep anemia, it is necessary to periodically transfusion of the erythrocyty mass, which can be carried out outpatient.