Chronic myeloid leukemia code according to MCB 10. Acute myeloid leukemia (acute myeloid leukemia). Symptoms and abnormalities

In acute myeloid leukemia, malignant transformation and uncontrolled proliferation of abnormally differentiated, long-lived progenitor cells of the myeloid series cause the appearance of blast cells in the circulating blood, the replacement of normal bone marrow with malignant cells.

ICD-10 code

C92.0 Acute myeloid leukemia

Symptoms and diagnosis of acute myeloid leukemia

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

The incidence of acute myeloid leukemia increases with age and is the most common leukemia in adults with a median age of onset of 50 years. Acute myeloid leukemia can develop as a secondary cancer after chemotherapy or radiation therapy for various types of cancer.

Acute myeloid leukemia includes a number of subtypes that differ from each other in morphology, immunophenotype, and cytochemistry. Based on the predominant cell type, 5 classes of acute myeloid leukemia are described: myeloid, myeloid-monocytic, monocytic, erythroid and megakaryocytic.

Acute promyelocytic leukemia is a particularly important subtype and accounts for 10-15% of all cases of acute myeloid leukemia. It occurs in the youngest group of patients (median age 31) and predominantly in a specific ethnic group (Hispanics). This variant often debuts with bleeding disorders.

Treatment of acute myeloid leukemia

The goal of initial therapy for acute myeloid leukemia is to achieve remission, and, unlike acute lymphoblastic leukemia, in acute myeloid leukemia, the response is achieved with fewer drugs. The basic regimen of induction of remission includes continuous intravenous infusion of cytarabine or cytarabine in high doses for 5-7 days; during this time, daunorubicin or idarubicin is administered intravenously for 3 days. Some regimens include 6-thioguanine, etoposide, vincristine, and prednisolone, but the effectiveness of these regimens is unclear. Treatment usually results in severe myelosuppression, infectious complications, and bleeding; it usually takes a long time before the bone marrow is restored. During this period, careful preventive and supportive therapy is vital.

In acute promyelocytic leukemia (APL) and some other variants of acute myeloid leukemia at the time of diagnosis, disseminated intravascular coagulation (DIC) may be present, aggravated by the release of procoagulants by leukemic cells. In acute promyelocytic leukemia with t (15; 17) translocation, the use of AT-RA (transretinoic acid) promotes the differentiation of blast cells and the correction of disseminated intravascular coagulation within 2-5 days; when combined with daunorubicin or idarubicin, this regimen can induce remission in 80-90% of patients with a long-term survival rate of 65-70%. Arsenic trioxide is also effective in acute promyelocytic leukemia.

After achieving remission, an intensification phase is carried out with these or other drugs; high-dose cytarabine regimens can increase the duration of remission, especially in patients under 60 years of age. Prophylaxis of central nervous system damage is usually not carried out, since with sufficient systemic therapy, central nervous system damage is a rare complication. The benefits of supportive care have not been demonstrated in intensively treated patients, but it may be beneficial in other situations. Extramedullary involvement is rare as an isolated recurrence.

MKB 10 or the international classification of all diseases of the 10th convocation has almost all short designations of known pathologies, including oncological ones. Leukemia, in short according to ICD 10, has two precise encodings:

S91- Lymphoid form.
C92- Myeloid form or myeloid leukemia.

But you also need to take into account the nature of the disease. For designation, a subgroup is used, which is written after the dot.

Lymphocytic leukemia

Encoding	Lymphoid leukemia
C 91.0	Acute lymphoblastic leukemia with T or B progenitor cells.
C 91.1	Lymphoplasmic form, Richter's Syndrome.
C 91.2	Subacute lymphocytic (code not used at this time)
C 91.3	Prolymphocytic B-cell
C 91.4	Hairy cell and leukemic reticuloendotheliosis
C 91.5	T-cell lymphoma or leukemia of adults with HTLV-1-associated parameter. Options: smoldering, acute, lymphomatoid, smoldering.
C 91.6	Prolymphocytic T-cell
C 91.7	Chronic of large granular lymphocytes.
C 91.8	Mature B-cell (Burkitt)
C 91.9	Unrefined form.

Myeloid leukemia

Includes granulocytic and myelogenous.

Codes	Myeloid leukemia
C 92.0	Acute myeloid leukemia (AML) with a low differentiation rate, as well as a form with maturation. (AML1 / ETO, AML M0, AML M1, AML M2, AML with t (8; 21), AML (without FAB classification) NOS)
C 92.1	Chronic form (CML), BCR / ABL-positive. Philadelphia chromosome (Ph1) is positive. t (9: 22) (q34; q11). With a blast crisis. Exceptions: unclassified myeloproliferative disease; atypical, BCR / ABL-negative; Chronic myelomonocytic leukemia.
C 92.2	Atypical chronic, BCR / ABL negative.
From 92.3	Myeloid sarcoma in which the neoplasm consists of immature atypical meleoilic cells. It also includes granulocytic sarcoma and chloroma.
C 92.4	Acute promyelocytic leukemia with parameters: AML M3 and AML M3 with t (15; 17).
From 92.5	Acute myelomonocytic with parameters AML M4 and AML M4 Eo with inv (16) or t (16; 16)
C 92.6	With an 11q23 abnormality and a variation of the MLL chromosome.
From 92.7	Other forms. The exception is hypereosinophilic syndrome or chronic eosinophilic.
C 92.8	With multilinear dysplasia.
From 92.9	Unsophisticated forms.

Causes

Recall that the exact reason for the development of blood cancer is not known. That is why it is so difficult for doctors to fight this ailment and prevent it. But there are a number of factors that can increase the chance of red fluid oncology.

Increased radiation
Ecology.
Poor nutrition.
Obesity.
Excessive use of medicines.
Excess weight.
Smoking, alcohol.
Harmful work associated with pesticides and chemicals that can affect hematopoietic function.

Symptoms and abnormalities

Anemia occurs as a result of inhibition of red blood cells, due to which oxygen does not reach healthy cells in full.
Severe and frequent headaches. It starts from stage 3, when intoxication occurs due to a malignant tumor. It can also be the result of advanced anemia.
Persistent colds and infectious and viral diseases with a long period. It happens when healthy leukocytes are replaced by atypical ones. They do not fulfill their function and the body becomes less protected.
Joint pain and withdrawal.
Weakness, fatigue, drowsiness.
Systematic low-grade fever for no reason.
Changes in smell, tastes.
Loss of weight and appetite.
Long bleeding with a decrease in the number of platelets in the blood.
Soreness, inflammation of the lymph nodes throughout the body.

Diagnostics

An accurate diagnosis can be made only after a thorough examination and passing a certain list of tests. Most often, people are caught on abnormal readings in biochemical and general blood tests.

For a more accurate diagnosis, a bone marrow puncture is performed from the pelvic bone. The cells are later sent for biopsy. Also, the oncologist conducts a complete examination of the body: MRI, ultrasound, CT, X-ray, to identify metastases.

Treatment, therapy and prognosis

The main type of treatment used is chemotherapy, when chemical poisons are injected into the bloodstream, which are aimed at destroying abnormal blood cells. The danger and ineffectiveness of this type of treatment is that healthy blood cells are also destroyed, of which there are so few.

If a primary focus is identified, the doctor may prescribe chemistry to completely destroy the bone marrow in this area. After the procedure, irradiation can also be carried out to destroy the remnants of cancer cells. In the process, stem cells are transplanted from a donor.

Frequency. 13.2 cases per 100,000 population among men and 7.7 cases per 100,000 population among women.

CLASSIFICATION
FAB classification(French American British) is based on the morphology of leukemic cells (structure of the nucleus, the ratio of the size of the nucleus and the cytoplasm). Acute myeloblastic (non-lymphoblastic) leukemia (AML) .. M0 - without cell maturation, myelogenous differentiation is proved only immunologically .. M1 - without cell maturation .. M2 - AML with cell differentiation, .. M3 - promyelocytic .. M4 - myelomonocytic .. M5 - monoblastic leukemia .. M6 - erythroleukemia .. M7 - megakaryoblastic leukemia. Acute lymphoblastic leukemia (ALL): .. L1 - without cell differentiation (morphologically homogeneous cells) .. L2 - with cell differentiation (morphologically heterogeneous population of cells) .. L3 - burkett-like leukemia. Undifferentiated leukemia - this category includes leukemias, the cells of which cannot be identified as myeloblastic or lymphoblastic (neither by chemical nor immunological methods). Myelopoietic dysplasia .. Refractory anemia without blastosis (in the bone marrow blasts and promyelocytes<10%) .. Рефрактерная анемия с бластозом (в костном мозге бласты и промиелоциты 10 30%) .. Рефрактерная анемия с избытком бластов в трансформации.. Хронический миеломоноцитарный лейкоз.

REAL classification(Revised Europian American classification of Lymphoid neoplasms), revised (European American) classification of lymphoid hematological malignancies. Pre B cell tumors .. Pre B lymphoblastic leukemia / lymphoma. Pre T cell tumors .. Pre T lymphoblastic leukemia / lymphoma. Tumors of peripheral B cells .. chronic lymphocytic leukemia / lymphoma from small lymphocytes .. Lymphoplasmacytic lymphoma .. Lymphoma from mantle cells .. Follicular lymphoma .. Lymphoma from cells of the marginal zone .. Hairy cell leukemia .. Plasmacytoma / plasmacytic myeloma .. Diffuse lymphoma from large lymphocytes .. Burkett's lymphoma. Tumors of peripheral T cells and NK cells .. T cell chronic lymphocytic leukemia .. Leukemia from large granular lymphocytes .. Fungal mycosis and Sesari syndrome T cell lymphoma .. Angioimmunoblastic T cell lymphoma .. Angiocentric lymphoma (lymphoma from NK and T cells) .. Intestinal T cell lymphoma .. Adult T cell leukemia / lymphoma .. Anaplastic large cell lymphoma

AML options(WHO classification, 1999). AML with t (8; 21) (q22; q22). AML with t (15; 17) (q22; q11 12). Acute myelomonoblastic leukemia. AML with pathological bone marrow eosinophilia (inv (16) (p13q22) or t (16; 16) (p13; q11). AML with 11q23 (MLL) defects. Acute erythroid leukemia. Acute megakaryocytic leukemia. Acute basophilic leukemia. Acute biphenotypic leukemias AML with multilinear dysplasia Secondary AML.

Immunohistochemical study(determination of the cellular phenotype) is necessary to clarify the immunological variant of leukemia, which affects the treatment regimen and clinical prognosis

... Acute lymphoblastic leukemia(247640, , somatic cell mutation) - 85% of all cases, accounting for up to 90% of all childhood leukemias.In adults, it develops quite rarely. Cytochemical reactions: positive for terminal deoxynucleotidyl transferase; negative for myeloperoxidosis, glycogen. The use of cell membrane markers made it possible to identify subspecies .. B - cellular - 75% of all cases .. With the absence of rosette formation .. T - cellular .. Other options (rarely). Differential diagnosis of subspecies is important for prognosis, because T - cell variants are difficult to treat.

... Acute myeloid leukemia more often occur in adults, the subtype depends on the level of cell differentiation. In most cases, a clone of myeloblasts comes from hematopoietic stem cells capable of multiple differentiation into colony-forming units of granulocytes, erythrocytes, macrophages or megakaryocytes, therefore, in most patients, malignant clones do not show signs of lymphoid or erythroid germs. AML is most often observed; has four variants (M0 - M3) .. M0 and M1 - acute leukemia without cell differentiation .. M2 - acute with cell differentiation .. M3 - promyelocytic leukemia, characterized by the presence of abnormal promyelocytes with giant granules; often combined with DIC, due to the thromboplastic effect of granules, which casts doubt on the advisability of using heparin in therapy. The prognosis for M3 is less favorable than for M0-M1 .. Myelomonoblastic and monoblastic leukemias (M4 and M5, respectively) are characterized by a predominance of non-erythroid cells of the monoblast type. M4 and M5 account for 5-10% of all AML cases. A common symptom is the formation of extra-bone marrow foci of hematopoiesis in the liver, spleen, gums and skin, hyperleukocytosis exceeding 50-100109 / l. The sensitivity to therapy and the survival rate are lower than in other variants of acute myeloid leukemia .. Erythroleukemia (M6). A variant of acute myeloid leukemia, accompanied by increased proliferation of erythroid precursors; the presence of abnormal blast nucleated erythrocytes is characteristic. The effectiveness of the treatment of erythroleukemia is similar to the results of therapy of other subtypes or somewhat lower. Megakaryoblastic leukemia (M7) is a rare variant, combined with bone marrow fibrosis (acute myelosclerosis). Poorly amenable to therapy. The prognosis is poor.
The pathogenesis is due to the proliferation of tumor cells in the bone marrow and their metastasis to various organs. The suppression of normal hematopoiesis is associated with two main factors:. damage and displacement of the normal hematopoiesis germ by poorly differentiated leukemic cells. the production of inhibitors by blast cells that inhibit the growth of normal hematopoietic cells.

Stages of acute leukemia... Primarily - the active phase. Remission (during treatment) - complete clinical - hematological .. The content of blasts in the bone marrow is less than 5% with normal cellularity .. There is no proliferative syndrome in the clinical picture. Relapse (early and late) .. Isolated bone marrow - the content of blasts in the bone marrow is more than 25% .. Extramedullary ... Neuroleukemia (neurological symptoms, cytosis of more than 10 cells, blasts in the cerebrospinal fluid) ... Testicular (an increase in the size of one or two testicles , the presence of blasts was confirmed by cytological and histological studies) .. Mixed. Terminal phase (in the absence of treatment and resistance to therapy)

Symptoms (signs)

The clinical picture of acute leukemia is determined by the degree of infiltration of the bone marrow by blast cells and inhibition of hematopoietic germs. Oppression of bone marrow hematopoiesis .. Anemic syndrome (myelophthisic anemia) .. Hemorrhagic syndrome (due to thrombocytopenia noted skin hemorrhages - petechiae, ecchymosis; bleeding from the mucous membranes - nosebleeds, internal bleeding) .. Infections (dysfunction of leukocytes). Lymphoproliferative syndrome .. Hepatosplenomegaly .. Swollen lymph nodes. Hyperplastic syndrome .. Bone pain .. Lesions of the skin (leukemides), meninges (neuroleukemia) and internal organs. Intoxication syndrome .. Weight loss .. Fever .. Hyperhidrosis .. Severe weakness.

Diagnostics

Diagnosis acute leukemia is confirmed by the presence of blasts in the bone marrow. To identify the subtype of leukemia, histochemical, immunological and cytogenetic research methods are used.

Laboratory research... In peripheral blood, the level of leukocytes can vary from severe leukopenia (below 2.0109 / l) to hyperleukocytosis; anemia, thrombocytopenia; the presence of blast cells up to total blastosis. Hyperuricemia due to an accelerated cell life cycle. Hypofibrinogenemia and increased content of fibrin degradation products due to concomitant DIC. The influence of drugs. HA should not be given until a definitive diagnosis has been made. The high sensitivity of blast cells to prednisolone leads to their destruction and transformation, which complicates the diagnosis.
Complex treatment; the goal is to achieve complete remission. Currently, hematology centers use various chemotherapy protocols based on the principles of polychemotherapy and treatment intensification.

. Chemotherapy consists of several stages .. Induction of remission ... In ALL - one of the schemes: a combination of intravenous vincristine weekly, oral prednisolone daily, daunorubicin and asparaginase for 1-2 months continuously ... With AML - a combination of intravenous cytarabine drip or s / c, daunorubicin IV, sometimes in combination with thioguanine. More intensive post-induction chemotherapy, destroying the remaining leukemia cells, increases the duration of remission .. Consolidation of remission: continuation of systemic chemotherapy and prevention of neuroleukemia in ALL (endolumbar administration of methotrexate in ALL in combination with radiation therapy to the brain with spinal cord capture) .. Supportive therapy: periodic courses of remission re-induction.

With AML M3, treatment with retinoic acid preparations (tretinoin) is carried out.
... Bone marrow transplantation is the method of choice for acute myeloid leukemia and for recurrence of all acute leukemias. The main condition for transplantation is complete clinical and hematological remission (the content of blasts in the bone marrow is less than 5%, the absence of absolute lymphocytosis). Before the operation, you can carry out chemotherapy in ultra-high doses, alone or in combination with radiation therapy (with the aim of completely destroying leukemic cells) .. The optimal donor is an identical twin or sibling; donors with a 35% match for Ag HLA are used more often. In the absence of compatible donors, autotransplantation of bone marrow taken during the period of remission is used. The main complication is the graft-versus-host reaction. It develops as a result of transplantation of donor T - lymphocytes, which recognize the recipient's Ag as foreign and cause an immune response against them. Acute reaction develops within 20-100 days after transplantation, delayed - after 6-12 months ... The main target organs are skin (dermatitis), gastrointestinal tract (diarrhea) and liver (toxic hepatitis) ... Treatment is long, usually limited prescribing combinations of prednisolone, cyclosporine and low doses of azathioprine .. The course of the post-transplant period is also influenced by preparatory treatment regimens, the development of interstitial pneumonia, and transplant rejection (rarely).

. Substitution therapy.. Transfusion of erythrocyte mass to maintain the Hb level not lower than 100 g / l. Transfusion conditions: unrelated donor, use of leukocyte filters .. Transfusion of fresh platelet mass (reduces the risk of bleeding). Indications: platelet count less than 20109 / l; hemorrhagic syndrome with platelet count less than 50109 / l.

. Prevention of infections- the main condition for the survival of patients with neutropenia resulting from chemotherapy .. Complete isolation of the patient .. Strict sanitary and disinfection regime - frequent wet cleaning (up to 4-5 r / day), ventilation and quartzing of the wards; use of disposable instruments, sterile clothing of medical personnel .. Prophylactic use of antibiotics, antifungal and antiviral drugs (if the content of segmented neutrophils is less than 0.5109 / L, prophylaxis of Pneumocystis pneumonia is indicated) ... When body temperature rises, clinical and bacteriological studies are carried out and immediately begin treatment with combinations of broad-spectrum bactericidal antibiotics: cephalosporins, aminoglycosides and semisynthetic penicillins ... With secondary rises in body temperature after treatment with broad-spectrum antibiotics, antifungal agents (amphotericin B) are empirically used. Colony-stimulating agents can be prescribed for the prevention and treatment of neutropenia. factors (for example, molgramostim).

Forecast. The prognosis for children with acute lymphocytic leukemia is good: 95% or more have complete remission. 70-80% of patients have no manifestations of the disease within 5 years, they are considered cured. If a relapse occurs, in most cases a second complete remission can be achieved. Patients with second remission are candidates for bone marrow transplantation with a long-term survival rate of 35-65%. The prognosis in patients with acute myeloid leukemia is unfavorable. 75% of patients receiving adequate treatment using modern chemotherapy regimens achieve complete remission, 25% of patients die (the duration of remission is 12-18 months). There are reports of a cure in 20% of cases with continued intensive care after remission. The prognosis for M3 - variant of AML improves with treatment with retinoic acid preparations. Patients under 30 years of age after reaching the first complete remission can undergo bone marrow transplantation. Long-term remission develops in 50% of young patients undergoing allogeneic transplantation. Encouraging results have also been obtained with autologous bone marrow transplants.

Age features
. Children.. 80% of all acute leukemias are ALL .. Adverse prognostic factors in ALL ... Child's age under 1 year old and over 10 years old ... Male sex ... T - cell variant of ALL ... Leukocyte count at the time of diagnosis more 20109 / L ... Lack of clinical and hematological remission against the background of induction .. Prognosis and course. 80% of the exit to clinical - hematological remission. 5 - year survival rate - 40-50%.

. Elderly... Reduced tolerance to allogeneic bone marrow. The maximum age for transplantation is 50 years. Autologous transplantation can be performed in patients over 50 years of age in the absence of organ damage and general somatic well-being.

Abbreviations... MDS - myelodysplastic syndrome. ALL - acute lymphoblastic leukemia. AML - acute myeloid leukemia.

ICD-10. C91.0 Acute lymphoblastic leukemia C92 Myeloid leukemia [myeloid leukemia] .. C93.0 Acute monocytic leukemia

Information: LEUKEMIA is a term that unites numerous tumors of the hematopoietic system arising from hematopoietic cells and affecting the bone marrow. The division of leukemias into two main groups - acute and chronic - is determined by the structure of tumor cells: acute leukemias, the cellular substrate of which is represented by blasts, and chronic leukemias, 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 a particular leukemia to the group of acute or chronic. Etiology, pathogenesis. The cause of acute leukemia and chronic myeloid leukemia in humans can be violations of the composition and structure of the chromosomal apparatus, hereditary 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 action of chemical mutagens. An increase in the frequency of acute leukemia among persons exposed to benzene, as well as among patients receiving cytostatic immunosuppressants (imuran, cyclophosphamide, leukaran, sarcolysin, mustargen, etc.); the frequency of acute leukemia among this contingent of patients increases hundreds of times. There are known facts of the emergence of acute myeloid leukemia, acute erythromyelosis against the background of long-term chemotherapy of chronic lymphocytic leukemia, Waldenstrom's macroglobulinemia, multiple myeloma, lymphogranulomatosis and other tumors. The role of hereditary defects in myeloid and lymphatic tissues predisposing to glucose is shown. Observations of the dominant and recessive inheritance of chronic lymphocytic leukemia are described, a low incidence of this leukemia in some ethnic groups and an increased incidence in others. More often in these cases, it is not the leukemia itself that is inherited, but increased variability - the instability of chromosomes, predisposing the parental myeloid or lymphatic cells to leukemic transformation. The use of chromosomal analysis made it possible to establish that with any leukemia, a clone of tumor leukemic cells, descendants of one initially mutated cell, is dispersed throughout the body. The instability of the genotype of malignant cells in leukemia causes the appearance of new clones in the initial tumor clone, among which the most autonomous clones are "selected" during the life of the organism, as well as under the influence of therapeutic agents. This phenomenon explains the progression of the course of leukemia, their departure from the control of cytostatics. Acute leukemias. According to morphological (mainly cytochemical) criteria, the following main forms of acute leukemia are distinguished: lymphoblastic, myeloblastic, promyelocytic, myelomonoblastic, monoblastic, megakaryoblastic, erythromyelosis, plasmablastic, undifferentiated, low percentage acute leukemia. All acute leukemias are characterized by growing "causeless" weakness, malaise, sometimes shortness of breath, dizziness caused by anemia. Increased body temperature, intoxication are common symptoms of non-lymphoblastic acute leukemia. Enlargement of lymph nodes, liver and spleen in the expanded stage does not occur in all acute leukemias, but it can develop regardless of the form of acute leukemia in the terminal stage. Frequent hemorrhagic syndrome, caused primarily by thrombocytopenia: bleeding of the mucous membranes, petechial rash on the skin, especially the legs. In the lungs, myocardium and other tissues and organs, leukemic blast infiltrates may appear. Diagnosis of acute leukemia is based on the data of cytological examination of blood and bone marrow, revealing a high percentage of blast cells. In the early stages, they are usually absent in the blood, but cytopenia is expressed. Therefore, in case of cytopenia, even involving one sprout, a bone marrow puncture is necessary, which can be done on an outpatient basis. In the bone marrow, there is a high (tens of percent) content of blasts in all acute leukemias, with the exception of acute low percent leukemia, in which for many months the percentage of blast cells in the blood and bone marrow may be less than 15-20, and in the bone marrow with this form as a rule, the percentage of blasts is less than in the blood. The form of acute leukemia is established using histochemical methods. The most common forms of acute leukemia in adults are myeloblastic and myelomonoblastic leukemia. At the onset of the disease in these forms, the liver and spleen are usually normal in size, the lymph nodes are not enlarged, however, deep granulocytopenia, anemia, and thrombocytopenia are not uncommon. Intoxication is often expressed, the body temperature is increased. Power cells have structural nuclei with a delicate network of chromatin, often several small nucleoles; the cytoplasm of blast cells contains azurophilic granularity or Auer corpuscles, which give a positive reaction to peroxidase and lipids. With myelomonoblastic leukemia, not only these substances are detected in the cytoplasm, but also alpha-naphthyl esterase, characteristic of the elements of the monocytic series; alpha-naphthyl esterase is inhibited by sodium fluoride. Acute lymphoblastic leukemia is more common in children. As a rule, from the very beginning it proceeds with lymphadenopathy, enlargement of the spleen, ossalgia. In the blood, at first, only moderate normochromic anemia, leukopenia can be noted, but in the bone marrow - total blastosis. Blast cells have a rounded nucleus with a delicate network of chromatin and 1–2 nucleoli, and a granular, narrow cytoplasm. During the PIC reaction, lumps of glycogen are detected in the cytoplasm, concentrated in the form of a necklace around the nucleus. Acute promyepocytic leukemia is quite rare; until recently, it was characterized by the rapidity of its current. It is characterized by severe intoxication, bleeding and hypofibrinogenemia caused by disseminated intravascular coagulation. Lymph nodes, liver, and spleen are usually not enlarged. In the hemogram, anemia, severe thrombocytopenia, in the bone marrow, a large percentage of atypical blasts. Power cells of various sizes and shapes have a cytoplasm, densely filled in some cells with large violet-brown granularity, located on the nucleus, in others, with fine abundant azurophilic granularity; Auer's little bodies are not uncommon. Granularity contains acidic sulfated mucopolysaccharides. The nuclei of these leukemic cells in the blood often have a two-lobed shape, even more often their shape is difficult to distinguish due to the abundance of granularity in the cytoplasm. The immediate cause of death of a patient is most often a cerebral hemorrhage. Acute monoblastic leukemia is relatively rare. The typical onset of this form differs little from myeloblastic, but intoxication and an increase in body temperature to febrile numbers are more pronounced. A common symptom is hyperplasia of the gingival mucosa due to leukemic proliferations in them. In the blood, at first, a granulocytic sprout can be relatively preserved, along with blast ones, many mature, more or less ugly monocytes are found. Power cells have a bean-shaped structural nucleus with several nucleoles and a grayish-blue cytoplasm, sometimes with scanty azurophilic granularity. Cytochemically, a positive reaction to alpha-naphthyl esterase, suppressed by sodium fluoride, a weakly positive reaction to peroxidase and lipids is revealed. In the blood serum and urine of these patients, the level of lysozyme is high. Acute plasmablastic leukemia is characterized by the appearance in the bone marrow and blood of plasmablasts and plasma cells with features of cellular atypism; in addition, many undifferentiated blasts are found. The characteristic cytochemical features of this form of acute leukemia are unknown; its feature is the detection of paraprotein in serum. Often expressed extramedullary leukemic foci - 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 nucleus, narrow cytoplasm with filamentous outgrowths), as well as undifferentiated blasts. Often, ugly megakaryocytes and fragments of their nuclei are found in the blood and bone marrow. Thrombocytosis is characteristic (more than 1000-lO (in the fourth degree) μl). Acute erythromyelosis is relatively rare. The disease is characterized by hyperplasia of red cells without signs of severe hemolysis. Clinical symptoms: progression of normo- or hyperchromic anemia without reticulocytosis (usually up to 2%), mild icterus due to the breakdown of erythrokaryocytes, increasing leukopenia and thrombocytopenia. In the bone marrow, the content of red cells with the presence of multinucleated erythroblasts and undifferentiated Power cells is increased. Unlike other forms of acute leukemia, red tumor cells often differentiate to the stage of an oxyphilic normocyte or to an erythrocyte. Acute erythromyelosis is often transformed into acute myeloid. Neuroleukemia is one of the most frequent complications of acute leukemia, less often chronic myeloid leukemia. Neuroleukemia is a leukemic lesion (infiltration) of the nervous system. This complication is especially common in acute lymphoblastic leukemia in children, less often in other forms of acute leukemia. The onset of neuroleukemia is due to the metastasis of leukemic cells into the membranes of the brain and spinal cord or into the substance of the brain (prognostically, this is a more severe type of tumor growth). The clinic of neuroleukemia consists of meningeal and hypertensive syndromes. Persistent headache, repeated vomiting, lethargy, irritability, swelling of the optic discs, nystagmus, strabismus and other signs of cranial nerve damage and meningeal signs are noted. There is a high blast cytosis in the cerebrospinal fluid. The detection of high cytosis and blast cells in the cerebrospinal fluid is an earlier sign of nairoleukemia than the described clinical picture. With intracerebral metastases - a picture of a brain tumor without cytosis. Treatment. In acute leukemia, urgent hospitalization is indicated. In some cases, with an accurate diagnosis, cytostatic treatment is possible on an outpatient basis. Pathogenetic treatment is used to achieve remission with the help of combined administration of cytostatics in order to eliminate all obvious and suspected leukemic foci, while severe depression of hematopoiesis is possible. Remission in acute leukemia is a condition in which the level of platelets in the blood is above 10 -104 in 1 μl, leukocytes are above 3,000 μl, in the bone marrow there are less than 5% of blasts, and lymphoid cells are less than 30%, there are no extra-marrow leukemic proliferates. In acute lymphoblastic leukemia in children, a mandatory criterion for completeness of remission is the normal composition of the cerebrospinal fluid. In children with acute lymphoblastic leukemia, the most effective combination of vincristine, prescribed at a dose of 1.4 mg / m2 (no more than 2 mg) once a week, intravenously, and prednisone orally daily at a dose of 40 mg / m2. With this therapy, remission is achieved in about 95% of children within 4-6 weeks. Already during the period of achieving remission, prevention of neuroleukemia begins: the first lumbar puncture should be done the next day after the diagnosis of acute lymphoblastic leukemia is established, and at the same time methotrexate (ametopterin) should be administered intralumbally at a dose of 12.5 mg / m2. Lumbar punctures with the introduction of methotrexate at the indicated dose are repeated every 2 weeks until remission is obtained. Immediately upon reaching remission, a special prophylactic course is carried out, including irradiation of the head at a dose of 2400 rad from bilateral fields with the capture of I and II cervical vertebrae, but with protection of the eyes, mouth, the entire area of the facial skull, and a simultaneous 5-fold (for 3 weeks of irradiation) Intralumbar administration of methotrexate at the same dose (12.5 mg / m2). When neuroleukemia is diagnosed during lumbar puncture, prophylactic irradiation of the head is canceled, neuroleukemia is treated with intra-lumbar administration of two cytostatic drugs: methotrexate at a dose of 10 mg / m2 (maximum 10 mg) and cytosar (an initial dose of 5 mg / m2 is gradually increased to 30 mg / m2). m2). During the period of remission of acute lymphoblastic leukemia, children undergo continuous cytostatic therapy with three cytostatics - 6-mercaptopurine (50 mg / m2 per day) daily, cyclophosphamide (200 mg / m2 once a week), methotrexate (20 mg / m2 once a week); treatment continues for 3.5-5 years. In acute lymphoblastic leukemia in adults and children with unfavorable baseline indications (late started and interrupted treatment before admission to therapy according to the program, age over 10-12 years, baseline leukocyte count more than 20,000 in 1 μL) in the first week of remission received according to the program , including vincristine, prednisolone and rubomycin, one of the cytostatic combinations is prescribed: COAP, or CHOP, or POMP. The combination of COAP consists of cyclophosphamide and cytosar, administered from the 1st to the 4th day of the IV course at 50 mg / m2 3 times a day with a syringe; vincristine administered at a dose of 1.4 mg / m2 IV on day 1 and prednisolone given daily from day 1 to day 4 at a dose of 100 mg / m2. The CHOP combination consists of cyclophosphamide administered intravenously at a dose of 750 mg / m2 on day 1 of the course, adriamycin - 50 mg / m2 intravenously on day 1, vincristine - 1.4 mg / m2 (maximum 2 mg ) on the 1st day IV and prednisolone given daily from the 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, including 6-mercaptopurine (purinethol) at 300-500 mg / m2 per day orally from day 1 to day 5, vincristine - 1.4 mg / m2 i.v. in the 1st day, methotrexate - 7.5 mg / m2 IV daily from the 1st to the 5th day and prednisone administered orally daily at 200 mg / m2 per day. One of these courses is carried out at the beginning of remission to consolidate it (consolidate). Then (after exiting cytopenia - raising the level of leukocytes to 3000 cells in 1 mm3), therapy for maintaining remission is started; in acute lymphoblastic leukemia, it is carried out continuously with the same three drugs (6-mercaptopurine, methotrexate and cyclophosphamide) as in children 2-10 years old, but every month and a half instead of this therapy, prescribed orally in tablets or, like cyclophosphamide, in powder, conduct alternately the course. COAP, CHOP or POMP (for the entire duration of maintenance therapy, i.e. for 5 pet, choose any two of these three courses for this patient). Regardless of age, patients with acute lymphoblastic leukemia are prevented from neuroleaemia with two cytostatic drugs: methotrexate (10 mg / m2, maximum 10 mg) and cytosar (in an increasing dose from 5 to 30 mg - only 5 intra-lumbar injections) or head irradiation (dose 24 Grza 15 sessions) and methotrexate administered intralumbally 5 times simultaneously with irradiation at a dose of 12.5 mg / m2. In acute non-lymphoblastic leukemia, the main drugs used to achieve remission are cytosar and rubomycin (or adriamycin). They can be prescribed in combination "7 + Z": Cytosar is administered continuously for 7 days at a daily dose of 200 mg / m2 or 2 times a day every 12 hours at 200 mg / m2 for 2 hours IV; rubomycin is administered intravenously with a syringe at a dose of 45 mg / m2 (30 mg / m2 for persons over 60 years old) on the 1st, 2nd and 3rd days of the course. 6-mercaptopurine can be added to cytosar and rubomycin, administered every 12 hours at a dose of 50 mg / m2, while the dose of cytosar is reduced to 100 mg / m2 administered every 12 hours. Cytosar is administered for 8 days, 6-mercaptopurine - from the 3rd to the 9th day. When remission is achieved, the fixing course - consolidation - may be the same as that leading to remission. To maintain remission, either the same combination of cytosar and rubomycin (course "7 + 3") is used, prescribed every month with an interval of 2.5 or 3 weeks, or 5-day administration of cytosar s / c at 100 mg / m2 every 12 hours in combination (on the first day of the course) with one of such cytostatics as cyclophosphamide (750 mg / m2) or rubomycin (45 mg / m2) or vincristine (1.4 mg / m2 on the 1st day) and prednisolone (40 mg / m2) m2 from the 1st to the 5th day) or methotrexate (30 mg / m2). Maintenance therapy is continued for 5 years, as in acute lymphoblastic leukemia. All patients receive prophylaxis of neuroleukemia. The first lumbar puncture with the introduction of methotrexate at a dose of 12.5 mg / m2 (maximum 15 mg) is performed for 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 prevention of neuroleukemia is carried out after achieving remission; in children with acute lymphoblastic leukemia, during the induction of remission, methotrexate is re-administered at a dose of 12.5 mg / m2 (maximum 15 mg) every 2 weeks. In case of reactions before administration, prednisolone is prescribed intravenously at a dose of 120 mg. Chronic leukemia. More common are lymphocytic leukemia, myeloid leukemia, multiple myeloma, erythremia, less often chronic subleukemic myelosis (osteomyelosclerosis, myelofibrosis), chronic monocytic leukemia, Waldenstrom's macroglobulinemia. In chronic myeloid leukemia, the tumor process affects both granulocytic and platelet and erythrocytic bone marrow growths. The progenitor of the tumor is the precursor cell of myelopoiesis. The process can spread to the liver, spleen, and in the terminal stage, any tissue can be affected. In the clinical course of chronic myeloid leukemia, the extended and terminal stages are distinguished. At the beginning of the expanded stage, the patient has no complaints, the spleen is not enlarged or slightly enlarged, the composition of the peripheral blood is changed. At this stage, the diagnosis can be established by analyzing the "unmotivated" nature of neutrophilic leukocytosis with a shift in the formula to myelocytes and promyelocytes, detecting a significantly increased leukocyte / erythrocyte ratio in the bone marrow and the "Philadelphia" chromosome in blood granulocytes and bone marrow cells. In the trepanate of the bone marrow, already during this period, as a rule, an almost complete displacement of fat by the myeloid tissue is observed. The extended stage can last an average of 4 years. With proper therapy, the condition of patients remains satisfactory, they remain able to work, lead a normal life with outpatient observation and treatment. In the terminal stage, the course of chronic myeloid leukemia acquires the features of malignancy: high fever, rapidly progressive exhaustion, bone pain, severe weakness, rapid enlargement of the spleen, liver, and sometimes swollen lymph nodes. This stage is characterized by the appearance and rapid growth of signs of suppression of normal hematopoietic growths - anemia, thrombocytopenia, complicated by hemorrhagic syndrome, granulocytopenia, complicated by infection, necrosis of the mucous membranes. The most important hematological sign of the terminal stage of chronic myeloid leukemia is a blast crisis - an increase in the content of blast cells in the bone marrow and blood (first, more often myeloblasts, then undifferentiated blasts). Karyologically, in the terminal stage, in more than 80% of cases, the appearance of aneuploid clones of hematopoietic cells containing an abnormal number of chromosomes is determined. The life expectancy of patients at this stage often does not exceed 6-12 months. Treatment of chronic myeloid leukemia is carried out from the moment of diagnosis. In the expanded stage, therapy with myelosan is effective at a dose of 2-4 mg / day (with a leukocyte level of more than 100,000 in 1 mm3, up to 6 mg / day is prescribed). Treatment is carried out on an outpatient basis. If myelosan is ineffective, myelobromol is prescribed (with significant splenomegaly, irradiation of the spleen can be performed). When the process enters the terminal stage, combinations of cytostatic drugs are used, which are usually used for the treatment of acute leukemia: vincristine and prednisolone, VAMP, cytosar and rubomycin. At the beginning of the terminal stage, myelobromol is often effective. Chronic lymphocytic leukemia is a benign tumor of the immune system; the basis of the tumor is formed by morphologically mature lymphocytes. The onset of the disease is often not possible to determine: in the midst of complete health and the absence of any unpleasant subjective sensations in the patient, a small, but gradually increasing lymphocytosis is found in the blood. In the early stages, the white blood cell count may be normal. A characteristic symptom of the disease is swollen lymph nodes. Sometimes their increase is found simultaneously with changes in the blood, sometimes later. An enlarged spleen is a common symptom; less often the liver enlarges. In the blood, along with an increase in the number of lymphocytes, the presence of single prolymphocytes and sometimes rare lymphoblasts, it is often possible to note the so-called Gumprecht shadows characteristic of chronic lymphocytic leukemia - lymphocyte nuclei destroyed during the preparation of a smear, in which nucleoles can be seen among the chromatin thyroids. In the advanced stage of the disease, the content of neutrophils, platelets and erythrocytes can remain at a normal level for many years. In the bone marrow in chronic lymphocytic leukemia, a high percentage of lymphocytes are found. The development of the disease is often accompanied by a decrease in the total level of gamma globulins. Suppression of humoral immunity is manifested by frequent infectious complications, especially pneumonia. Another common complication is cytopenia, more often anemia and thrombocytopenia. This complication may be associated with the emergence of autoantibodies against erythrocytes and platelets or against erythrokaryocytes and megakaryocytes. But this is not the only mechanism of cytopenia in chronic lymphocytic leukemia; possibly suppressive effect of lymphocytes (in particular, T-lymphocytes) on progenitor cells of erythropoiesis or thrombocytopoiesis. The terminal stage of chronic lymphocytic leukemia, manifested by sarcoma growth or blast crisis, is rarely observed, especially a blast crisis. The development of lymphosarcoma in some cases may be accompanied by a change in blood lymphocytosis by neutrophilia. Hairy cell leukemia is a special form of chronic lymphocytic leukemia, in which lymphocytes have a homogeneous nucleus resembling a blast nucleus, villous outgrowths of the cytoplasm. The cytoplasm of these cells contains a lot of acid phosphatase, which is resistant to the action of tartaric acid. The clinical picture is characterized by an enlargement of the spleen, a slight increase in peripheral lymph nodes and severe cytopenia. In 75% of cases of hairy cell leukemia, proceeding with an enlargement of the spleen, splenectomy is effective. If cytopenia is not associated with an enlargement of the spleen or there are any other organ changes or lymphadenopathy, the therapy of choice is the use of interferon alpha (3,000,000-9,000,000 IU / m daily for many months, taking into account the positive dynamics of blood counts, changes in A separate form is chronic lymphocytic leukemia with skin lesions - the Cesari form.The process often begins with skin lesions, pruritus, the appearance of local lymphatic infiltrates under the epidermis, which can then become total.Lymphocytosis and the percentage of ugly lymphocytes in the blood gradually increase. These are usually large cells with indented contours of the nucleus of the looped structure, but the cells can also be small with a bean-shaped nucleus. It has been proven that these lymphocytes belong to T cells. Lymphadenopathy can be of a mixed nature: some lymph nodes are enlarged reactively due to infection in the skin, others - due to with their leukemic infiltration. The spleen may become develop in the process of illness. In the treatment of the Cesari form, long-term use of small doses of chlorbutin often gives an effect (2-4 mg / day daily for several months under the control of blood tests, primarily platelet count - 1 time in 2-3 weeks), which relieves itching, reduces leukemia infiltration of the skin. Treatment of chronic lymphocytic leukemia, manifested by an increase in leukocytosis, moderate lymphadenopathy, begins with the use of chlorobutin. With a significant size of the lymph nodes, cyclophosphamide is used. Steroid therapy is prescribed for autoimmune complications, hemorrhagic syndrome, as well as the ineffectiveness of certain cytostatics (in the latter case, chlorbutin or cyclophosphamide is sometimes combined with prednisolone). Long-term use of steroids in chronic lymphocytic leukemia is contraindicated. With a significant density of peripheral lymph nodes, involvement of the abdominal lymph nodes in the process, combinations of drugs such as VAMP or a combination of cyclophosphamide, vincristine or vinblastine and prednisone (COP or CVP) are successfully used. The spleen, lymph nodes, and skin are irradiated. One of the methods of treating autoimmune cytopenia in chronic lymphocytic leukemia is splenectomy. Treatment of infectious complications is of particular importance. Recently, leukocytopheresis has been used to treat lymphocytic leukemia with high leukocytosis and cytopenia. Patients with chronic lymphocytic leukemia maintain good health and ability to work for many years. Chronic monoritic leukemia refers to rare forms of leukemia, characterized by high monocytosis in the peripheral blood (20-40%) with a normal or slightly increased number of leukocytes. Along with mature monocytes, there are single promonocytes in the blood. In the bone marrow, the percentage of monocytes is slightly increased, but in the trepanate, hyperplasia of the bone marrow tissue with diffuse proliferation of monocytic elements is observed. In the blood and urine, a high content of lysozyme. In 50% of patients, the spleen is palpable. The long-term successful course of chronic monocytic leukemia can be replaced by a terminal stage, which has the same features as the terminal stages of chronic myeloid leukemia. In the expanded stage, the process does not require special treatment; only with deep anemia, periodic transfusion of erythrocyte mass is necessary, which can be performed on an outpatient basis.