Subleukemic myelosis refers to leukemias, manifested by slightly increased polymorphic-cell myeloproliferation such as panmyelosis or myelomegakaryocytic myelosis, progressive myelofibrosis and osteomyelosclerosis, splenomegaly, hepatomegaly with three-growth myeloid metaplasia in these organs and, much less often, in other tissues.
What provokes Subleukemic myelosis:
In the literature, there were no data on the structure of the incidence of subleukemic myelosis.
Pathogenesis (what happens?) During Subleukemic myelosis:
Some researchers believe that in subleukemic myelosis, the process of hematopoiesis is primarily impaired at the level of the myelopoiesis progenitor cell. Its belonging to hemoblastoses and the secondary nature of myelofibrosis are based on studies of the types of G-6-PD in blood cells and fibroblasts of the bone marrow and skin in mulatto women heterozygous for this enzyme. According to one concept, myelofibrosis in this form of leukemia is caused by megakaryocytes and platelets, which produce a growth factor that enhances the proliferation of fibroblasts. The topography of myelofibrosis corresponds to the areas of accumulation of megakaryocytes. Supporters of subleukemic myelosis belonging to leukemia point to myeloid metaplasia in the spleen and other organs, the final exacerbation of the process as a power crisis, the presence of a malignant form of the disease and the sensitivity of such patients to cytostatic therapy.
Symptoms of Subleukemic Myelosis:
With a benign variant of subleukemic myelosis, a detailed clinical picture is preceded by a long asymptomatic period. Life expectancy from the moment of establishing the diagnosis ranges from 1.5 to 5 years, there are cases of a longer course of the disease (15-20 years or more).
Malignant forms of subleukemic myelosis are characterized by an acute (subacute) or fulminant course, early onset of a power crisis, deep thrombocytopenia, and severe hemorrhagic syndrome leading to death. Infectious complications, heart and liver failure, and thrombosis are common. In 10-17% of cases, portal hypertension with varicose veins of the esophagus is diagnosed.
The approximate wording of the diagnosis:
- Subleukemic myelosis; a favorable variant with a slow increase in the size of the spleen and liver, an increase in anemia, the number of leukocytes, platelets and the development of myelofibrosis.
- Subleukemic myelosis; acutely flowing variant with a pronounced enlargement of the spleen and liver, early development of a power crisis, anemia, deep thrombocytopenia with hemorrhagic syndrome (brain, nose and gingival bleeding), myelofibrosis.
Subleukemic myelosis is more commonly found in individuals over 40 years of age. Sometimes, for many years, patients do not notice any signs of illness, they go to the doctor with complaints of weight loss, recurrent fever, pain in the bones and in the spleen area. Against the background of insolvency of hemostasis and thrombocytopenia, hemorrhages occur in the skin, joints, often bleeding from the veins of the esophagus and stomach. Anemia is more often normochromic, rarely megaloblastic or hemolytic. In some cases, erythrocytosis and an increase in erythropoiesis in the bone marrow are detected. In the hemogram, the number of leukocytes is increased, sometimes reduced, neutrophilia is noted with a shift to the left. The platelet count is increased or normal, they are functionally defective. In the myelogram - megakaryocytosis (immature forms). In the bone marrow - narrowing of the cavities filled with fibrous tissue. In the enlarged spleen, liver and other organs and tissues, there are foci of extra-cerebral hemopoiesis of a polymorphic composition.
Diagnostics of the Subleukemic myelosis:
The diagnosis of subleukemic myelosis is established on the basis of clinical data and the results of a study of the state of hematopoiesis (hemogram, myelogram, bone marrow biopsy).
Subleukemic myelosis is differentiated from chronic myeloid leukemia, which occurs with subleukemic leukocytosis. The detection of the Ph "chromosome is a strong argument in favor of myeloid leukemia.
Differential diagnosis should also be carried out between subleukemic myelosis and secondary myelofibrosis, which can develop in malignant neoplasms, prolonged infections (tuberculosis), as well as in toxic effects (benzene and its derivatives, etc.).
Treatment for Subleukemic Myelosis:
In the early stages of subleukemic myelosis with moderate anemia and splenomegaly that does not cause abdominal discomfort, cytostatic treatment should not be used; you can limit yourself to general strengthening therapy. Indications for the appointment of cytostatics are splenomegaly with compression syndrome and symptoms of hypersplenism, thrombocythemia with the threat of thrombosis, progressive blastemia, pletora.
Myelobromol appoint 250 mg / day with an initial leukocyte count of at least 15-20 * 10 9 / l and a normal platelet count, a course dose of 4-10 g. With a slightly smaller number, glucocorticoid and anabolic hormones are prescribed for 7-14 days ... The drug is canceled when leukocytes reach 6-7 * 10 9 / l, and platelets - 100-150 * 10 9 / l.
Cyclophosphamide, the antitumor effect of which is less pronounced than myelobromol, is prescribed - in cases of a reduced number of leukocytes and platelets - 200-400 mg / day intravenously at intervals of 1-3 days (course dose 10-12 g) in combination with glucocorticoid hormones. In a blast crisis, the principles of treatment of acute leukemia are used.
The main clinical, hematological and radiological changes in subleukemic myelosis
The size of the spleen, liver | Splenomegaly, often the lower edge of the spleen reaches the small pelvis, hepatomegaly in 50% of patients (these symptoms may be absent), symptoms of abdominal discomfort |
Erythropoiesis | Anemnia, more often normochromic, sometimes megaloblastic or hemolytic in nature (decrease in the life span of erythrocytes, increase in the level of free bilirubin in the blood serum); in some cases, erythrocytosis, often aniso- and poikilocytosis, target-shaped and pear-shaped forms of erythrocytes, erythro- and normoblasts, reticulocytosis; in the bone marrow, erythropoiesis is sometimes enhanced |
Leukopoiesis | In the hemogram, the number of leukocytes is increased, but not significantly, rarely reduced; neutrophilia with a shift to the left, sometimes myeloblasts are found. The number of immature forms of neutrophils is increased in the bone marrow |
Thrombopoiesis | The number of platelets is increased in 50% of patients, they are functionally defective (reduced retraction of the blood clot, factor 3 level, platelet adhesiveness, increased bleeding time); the number of megakaryocytes, including immature forms, is increased in the bone marrow |
Extra medullary hematopoiesis | The presence of foci of three-growth hematopoiesis, consisting of cells of varying degrees of maturity, in the spleen, liver, and other organs is characteristic. |
Histological examinations | Massive growth of bone tissue with a decrease in the volume of active bone marrow and with a narrowing of its cavities filled with fibrous tissue, fat cells; bone beams are thickened, irregular in shape due to stratification of atypical bone tissue, osteoid |
X-ray data | On radiographs of bones (pelvis, vertebrae, ribs, skull, long tubular) the cortical layer is thickened, the normal trabecular structure is lost, obliteration of the bone marrow cavities can be detected |
Radiation therapy on the area of the sharply enlarged spleen causes a short-term positive effect, stopping the phenomena of abdominal discomfort, however, the development of deep cytopenia is possible.
Splenectomy indicated mainly in cases of deep hemolytic crises that are not amenable to drug therapy, with the threat of rupture of the spleen and recurrent heart attacks, with severe hemorrhagic thrombocytopenic syndrome. Splenectomy is contraindicated in the terminal stage, with thrombocytosis and hypercoagulability.
Glucocorticoid hormones prescribed for hemolytic anemia, cytopenias, prolonged fever of non-infectious origin, arthralgia. Anabolic hormones (nerobol, retabolil) are indicated for anemia caused by insufficiency of erythropoiesis, long-term treatment with glucocorticoid hormones. With deep anemia, erythrocyte mass transfusion is used; thrombocytopenic hemorrhagic syndrome is an indication for thrombocytopenic concentrate transfusions. With iron deficiency anemia, iron supplements are prescribed.
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 myeloid (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 differentiation of cells (morphologically homogeneous cells) .. L2 - with differentiation of cells (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 (blasts and promyelocytes in the bone marrow<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 leukemia In adults, it develops quite rarely. Cytochemical reactions: positive for terminal deoxynucleotidyl transferase; negative for myeloperoxidosis, glycogen. The use of markers of the cell membrane made it possible to isolate subspecies .. B - cellular - 75% of all cases .. With the absence of rosette formation .. T - cellular .. Other variants (rarely). Differential diagnosis of subspecies is important for prognosis, because T - cell variants are difficult to treat.
... Acute myeloid leukemia occur more often 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 frequent symptom is the formation of extraboneous foci of hematopoiesis in the liver, spleen, gums and skin, hyperleukocytosis exceeding 50-100109 / 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 for other subtypes or slightly lower. Megakaryoblastic leukemia (M7) is a rare variant associated with bone marrow fibrosis (acute myelosclerosis). Poorly amenable to therapy. The prognosis is unfavorable.
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 a 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 2.0109 / 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 prednisone 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 (tretinoin) preparations 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 ultrahigh 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 35% coincidence for Ag HLA are used more often. In the absence of compatible donors, autotransplantation of bone marrow taken during remission is used. The main complication is the graft-versus-host reaction. It develops as a result of transplantation of donor T - lymphocytes, recognizing the recipient's Ar as foreign and causing 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-term, 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 20109 / l; hemorrhagic syndrome with platelet count less than 50109 / 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), airing and quartzing the wards; use of disposable instruments, sterile clothing for medical personnel .. Prophylactic use of antibiotics, antifungal and antiviral drugs (if the content of segmented neutrophils is less than 0.5109 / L, prevention of Pneumocystis pneumonia is indicated) ... When body temperature rises, clinical and bacteriological studies are carried out and immediately start treatment with combinations of broad-spectrum bactericidal antibiotics: cephalosporins, aminoglycosides and semisynthetic penicillins ... In case of 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 .. Unfavorable prognostic factors in ALL ... Child's age under 1 year and over 10 years ... Male sex ... T - cell variant of ALL ... Leukocyte count at the time of diagnosis more 20109 / l ... Absence 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 is 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
The disease is often asymptomatic, as detected by routine clinical blood tests. CML can present with malaise, subfebrile fever, gout, increased susceptibility to infections, anemia, thrombocytopenia with bleeding (although platelet counts may also be elevated). Splenomegaly is also noted.
CML is often divided into three phases based on clinical characteristics and laboratory findings. In the absence of treatment, CML usually begins in a chronic phase, progresses over several years to an acceleration phase, and ultimately develops a blast crisis. Blast crisis is the terminal phase of CML, clinically similar to acute leukemia. One of the factors in the progression from chronic phase to blast crisis is the acquisition of new chromosomal abnormalities (in addition to the Philadelphia chromosome). Some patients by the time of diagnosis may already be in the acceleration phase or in a blast crisis.
About 85% of patients with CML are in the chronic phase at the time of diagnosis. During this phase, clinical manifestations are usually absent or there are “mild” symptoms such as malaise or a feeling of fullness in the abdomen. The duration of the chronic phase is different and depends on how early the disease was diagnosed, as well as on the treatment carried out. Ultimately, in the absence of effective treatment, the disease enters the acceleration phase.
Acceleration phase.
The diagnostic criteria for entering the acceleration phase can vary: the most widely used criteria are those established by researchers at the Anderson Cancer Center at the University of Texas, Sokal et al., And the World Health Organization. The WHO criteria are probably the most widely used, and distinguish the acceleration phase as follows:
10-19% of myeloblasts in the blood or bone marrow.
> 20% of basophils in the blood or bone marrow.
<100,000 тромбоцитов, вне связи с терапией.
> 1,000,000 regardless of therapy.
Cytogenetic evolution with the development of new abnormalities in addition to the Philadelphia chromosome.
Progression of splenomegaly or an increase in the number of leukocytes, regardless of therapy.
The acceleration phase is assumed if any of the above criteria is met. The acceleration phase indicates the progression of the disease and the expected blast crisis.
Blast crisis.
Blast crisis is the final stage of CML development, proceeding like acute leukemia, with rapid progression and short survival. A blast crisis is diagnosed based on one of the following signs in a patient with CML:
> 20% of myeloblasts or lymphoblasts in the blood or bone marrow.
Large groups of blasts in the bone marrow on biopsy.
Development of chloroma (solid focus of leukemia outside the bone marrow).
LEUKEMIA
Acute leukemia.
Chronic lymphocytic leukemia.
Chronic myeloid leukemia.
Polycythemia vera.
ACUTE LEUKEMIA
Definition.
Acute leukemia is a myeloproliferative tumor, the substrate of which is blasts, which are deprived of the ability to differentiate into mature blood cells.
ICD10: C91.0 - Acute lymphoblastic leukemia.
C92.0 - Acute myeloid leukemia.
C93.0 - Acute monocytic leukemia.
Etiology.
Latent viral infection, predisposing heredity, exposure to ionizing radiation can cause somatic mutations in the hematopoietic tissue. Among the mutant pluripotent cells close to the stem cell, a clone that is insensitive to immunoregulatory influences can form. A mutant clone forms an intensively proliferating and metastatic tumor outside the bone marrow, consisting of blasts of the same type. A distinctive feature of tumor blasts is the inability to further differentiate into mature blood cells.
Pathogenesis.
The most important link in the pathogenesis of acute leukemia is the competitive metabolic suppression by abnormal blasts of the functional activity of normal hematopoietic tissue and its displacement from the bone marrow. As a result, aplastic anemia, agranulocytosis, thrombocytopenia with characteristic hemorrhagic syndrome, severe infectious complications due to deep disturbances in all links of immunity, deep dystrophic shifts in the tissues of internal organs occur.
According to the FAB classification (cooperative group of hematologists of France, America and Britain, 1990), there are:
Acute lymphoblastic (lymphoid) leukemias.
Acute non-lymphoblastic (myeloid) leukemias.
Acute lymphoblastic leukemias are classified into 3 types:
L1 - acute microlymphoblastic type. Blast antigenic markers correspond to null ("neither T, nor B") or thymus-dependent (T) lymphopoiesis lines. It occurs mainly in children.
L2 - acute lymphoblastic. Its substrate is typical lymphoblasts, the antigenic markers of which are the same as in type L1 acute leukemia. More common in adults.
L3 - acute macrolimphocytic and prolymphocytic leukemia. Blasts have antigenic markers of B-lymphocytes, morphologically similar to Burkitt's lymphoma cells. This type is rare. It has a very poor prognosis.
Acute non-lymphoblastic (myeloid) leukemias are divided into 6 types:
M0 - acute undifferentiated leukemia.
M1 - acute myeloid leukemia without cell maturation.
M2 - acute myeloid leukemia with signs of cell maturation.
M3 - acute promyelocytic leukemia.
M4 - acute myelomonoblastic leukemia.
M5 - acute monoblastic leukemia.
M6 - acute erythromyelosis.
The clinical picture.
In the clinical course of acute leukemia, the following stages are distinguished:
Initial period (primary active stage).
In most cases, it begins acutely, often in the form of "flu". The body temperature suddenly rises, chills, sore throat, arthralgia, pronounced general weakness appear. Less often, the disease may first manifest itself with thrombocytopenic purpura, recurrent nose, uterine, and gastric bleeding. Sometimes OB begins with a gradual deterioration of the patient's condition, the appearance of mild arthralgias, bone pain, bleeding. In isolated cases, asymptomatic onset of the disease is possible.
In many patients, in the initial period of OB, an increase in peripheral lymph nodes and moderate splenomegaly are revealed.
Stage of advanced clinical and hematological manifestations (first attack).
It is characterized by a sharp deterioration in the general condition of patients. Typical complaints are severe general weakness, high fever, pain in the bones, in the left hypochondrium in the spleen area, bleeding. At this stage, clinical syndromes typical for OB are formed:
Hyperplastic (infiltrative) syndrome.
Enlargement of lymph nodes and spleen is one of the most typical manifestations of leukemic tumor dissemination. Leukemic infiltration often causes subcapsular hemorrhages, heart attacks, and ruptures of the spleen.
The liver and kidneys are also enlarged due to leukemic infiltration. Leukemic filtrates in the lungs, pleura, mediastinal lymph nodes are manifested by symptoms of pneumonia, exudative pleurisy.
Leukemic infiltration of the gums with swelling, hyperemia, and ulceration is common in acute monocytic leukemia.
Localized tumor masses (leukemides) in the skin, eyeballs, and in other places occur in non-lymphoblastic (myeloid) forms of leukemia in the later stages of the disease. In some myeloid leukemias, leukemides may have a greenish color ("chloroma") due to the presence of myeloperoxidase in the blast cells of the tumor.
Anemic syndrome.
Leukemic infiltration and metabolic inhibition of normal growths of bone marrow hematopoiesis lead to the occurrence of aplastic anemia. Usually normochromic anemia. In acute erythromyelosis, it can have a hyperchromic megaloblastoid character with a moderately pronounced hemolytic component. With severe splenomegaly, hemolytic anemia may occur.
Hemorrhagic syndrome.
It is caused by thrombocytopenia, disseminated intravascular coagulation syndrome. It is manifested by subcutaneous hemorrhages (thrombocytopenic purpura), bleeding gums, nasal, uterine bleeding. Possible gastrointestinal, pulmonary bleeding, gross hematuria. Along with hemorrhages, thrombophlebitis, thromboembolism, and other hypercoagulable disorders caused by disseminated intravascular coagulation often occur. This is one of the characteristic manifestations of acute promyelocytic and myelomonoblastic leukemia.
Immunodeficiency Syndrome.
The formation of an immunodeficiency state is due to the displacement of normal clones of immunocompetent cells from the bone marrow by leukemic blasts. Clinically manifested by fever, often of the hectic type. There are foci of chronic infection of different localization. Characterized by the occurrence of necrotizing ulcers, peritonsillar abscesses, necrotizing gingivitis, stomatitis, pyoderma, perrectal abscesses, pneumonia, pyelonephritis. Generalization of infection with the development of sepsis, multiple abscesses in the liver, kidneys, hemolytic jaundice, DIC is often the cause of the patient's death.
Neuroleukemia syndrome.
It is characterized by the metastatic spread of foci of blast proliferation into the meninges, the substance of the brain, structures of the spinal cord, and nerve trunks. It is manifested by meningeal symptoms - headache, nausea, vomiting, visual impairment, stiff neck muscles. The formation of large tumor-like leukemic infiltrates in the brain is accompanied by focal symptoms, cranial nerve paralysis.
Remission achieved as a result of the treatment.
Under the influence of the treatment, all clinical manifestations of the disease fade away (incomplete remission) or even complete disappearance (complete remission).
Relapse (second and subsequent attacks).
As a result of ongoing mutations, a clone of tumor blasts emerges that can "evade" the effects of cytostatic drugs used for maintenance treatment. An exacerbation of the disease occurs with the return of all syndromes typical for stages of advanced clinical and hematological manifestations of OB.
Under the influence of anti-relapse therapy, remission can be achieved again. Optimal treatment tactics can lead to recovery. In case of insensitivity to the treatment, OB passes into the terminal stage.
Recovery.
The patient is considered recovered if complete clinical and hematological remission persists for more than 5 years.
Terminal stage.
It is characterized by insufficient 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, internal organs by leukemic blasts, the system of normal hematopoiesis is totally suppressed, infectious immunity disappears, and deep disturbances in the hemostasis system occur. Death occurs from disseminated infectious lesions, intractable bleeding, severe intoxication.
Clinical features of the morphological types of acute leukemia.
Acute undifferentiated leukemia (M0). It is rare. It progresses very quickly with aggravation of severe aplastic anemia, pronounced hemorrhagic syndrome. Remissions are rare. Average life expectancy is less than 1 year.
Acute myeloid leukemia (M1-M2). The most common variant of acute non-lymphoblastic leukemia. Adults are more likely to get sick. Differs in a severe, stubbornly progressive course with severe anemic, hemorrhagic, immunosuppressive syndromes. Characterized by ulcerative necrotic lesions of the skin, mucous membranes. It is possible to achieve remission in 60-80% of patients. Average life expectancy is about 1 year.
Acute promyelocytic leukemia (M3). One of the most malignant variants. It is characterized by a pronounced hemorrhagic syndrome, which most often leads the patient to death. Violent hemorrhagic manifestations are associated with disseminated intravascular coagulation, which is caused by an increase in thromboplastin activity of leukemic promyelocytes. Their surface and cytoplasm contain 10-15 times more thromboplastin than normal cells. Timely treatment makes it possible to achieve remission in almost every second patient. The average life expectancy reaches 2 years.
Acute myelomonoblastic leukemia (M4). The clinical symptoms of this form of the disease are close to acute myeloid leukemia. The differences are in a greater tendency to necrosis. DIC syndrome occurs more often. Every tenth patient has neuroleukemia. The disease progresses rapidly. Serious infectious complications often occur. The average life expectancy and the frequency of stable remissions are two times less than in acute myeloid leukemia.
Acute monoblastic leukemia (M5). A rare form. In terms of clinical manifestations, it differs little from myelomonoblastic leukemia. Differs in a greater tendency to rapid and persistent progression. Therefore, the average life expectancy of patients with this form of leukemia is even less - about 9 months.
Acute erythromyelosis (M6). Rare forms. A distinctive feature of this form is persistent, profound anemia. Hyperchromic anemia with symptoms of mild hemolysis. In leukemic erythroblasts, megaloblastoid abnormalities are detected. Most cases of acute erythromyelosis are 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. It is accompanied by an increase in peripheral lymph nodes, spleen, liver. Hemorrhagic syndrome, necrotic ulcerative complications are rare. Life expectancy in acute lymphoblastic leukemia is from 1.5 to 3 years.
MINISTRY OF HEALTH OF THE IRKUTSK REGION
ORDER
ON THE APPROVAL OF THE AMBULATORY STANDARD FOR PROVIDING MEDICAL CARE IN SUBLEUKEMIC MYELOSIS
In order to improve the quality of medical care to the population of the Irkutsk region, in accordance with the Fundamentals of the legislation of the Russian Federation on the protection of the health of citizens of the Russian Federation, guided by paragraph 9 of the Regulation 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 of care for subleukemic myelosis.
2. This order is subject to official publication in the Oblastnaya newspaper.
3. Control over the execution of the order shall be entrusted to the head of the medical aid organization department of the Ministry of Health of the Irkutsk Region, L.L. Gavrilova.
The minister
G.M. Gaidarov
Application. AMBULATORY STANDARD FOR PROVIDING MEDICAL CARE FOR SUBLEUKEMIC MYELOSIS
Appendix to the order
ministries of health
Irkutsk region
1. PATIENT MODEL
Nosological form: subleukemic myelosis.
ICD-10 code: D47.1
Phase: any.
Stage: any.
Complications: regardless of complications.
Terms of delivery: outpatient and polyclinic care.
1.1. DIAGNOSTICS
Name | Frequency | The average |
|
Collection of anamnesis and complaints in case of diseases | |||
Visual examination at | |||
Palpation for organ diseases | |||
Study of the level of erythrocytes | |||
Study of the level of leukocytes | |||
Study of the level of platelets | |||
The ratio of leukocytes in the blood | |||
Viewing a blood smear for analysis | |||
Study of the level of reticulocytes | |||
Determination of the color index | |||
Study of the level of general | |||
Alkaline level research | |||
Study of the level of total protein in | |||
Level research | |||
Level research | |||
Sodium level study | |||
Serum potassium test | |||
Calcium study | |||
Study of creatinine levels | |||
Study of the level of uric acid | |||
Study of iron levels | |||
Level research | |||
Study of the level of urea | |||
Serological reaction to various | |||
Identification of tumor genes | |||
Obtaining a cytological preparation | |||
Calculation of the bone marrow formula | |||
Study of the chromosomal apparatus | |||
Obtaining a histological | |||
Histological examination | |||
Ultrasound examination of the liver | |||
Ultrasound procedure | |||
Cytochemical research | |||
Immunophenotyping of cells |
2.1. 365 DAY TREATMENT
Pharmacotera singing group | ATX group * | International non-proprietary name | Assignment frequency | ||
Antineoplastic, immunosuppressive and concomitant drugs Hydroxycarbamide | |||||
Interferon alpha-2 | 548 million IU |
||||
Prednisone | |||||
Antianemic agents: | 40,000 units | 2080000 UNITS |
|||
Disaggregants: | |||||
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