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

ICD-10 code

C92.0 Acute myeloid leukemia

Symptoms and diagnosis of acute myeloblastic leukemia

Symptoms include fatigue, pallor, fever, infection, bleeding, and easy subcutaneous hemorrhages; 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 the disease 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 have been 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 years) and predominantly in a specific ethnic group (Hispanics). This option often debuts with blood clotting disorders.

Treatment of acute myeloblastic leukemia

The goal of initial therapy for acute myeloid leukemia is to achieve remission, and unlike acute lymphoblastic leukemia, response in acute myeloid leukemia is achieved using fewer drugs. The basic remission induction regimen includes a continuous intravenous infusion of cytarabine or high-dose cytarabine 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 treatment regimens is unclear. Treatment usually results in severe myelosuppression, infectious complications, and bleeding; It usually takes a long time for the bone marrow to recover. During this period, careful preventative and supportive care is vital.

In acute promyelocytic leukemia (APL) and some other variants of acute myeloid leukemia, disseminated intravascular coagulation (DIC) may be present at the time of diagnosis, aggravated by the release of procoagulants by leukemia cells. In acute promyelocytic leukemia with translocation t (15; 17), the use of AT-RA (transretinoic acid) promotes the differentiation of blast cells and 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 long-term survival of 65-70%. Arsenic trioxide is also effective in acute promyelocytic leukemia.

After achieving remission, an intensification phase with these or other drugs is carried out; High-dose cytarabine regimens may prolong the duration of remission, especially in patients under 60 years of age. Prevention of central nervous system damage is usually not carried out, since central nervous system damage is a rare complication with sufficient systemic therapy. In intensively treated patients, no benefit has been demonstrated from maintenance therapy, but it may be useful in other situations. Extramedullary lesions as an isolated recurrence are rare.

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

CLASSIFICATION
FAB classification(Franco-American-British) is based on the morphology of leukemic cells (structure of the nucleus, ratio of the sizes of the nucleus and cytoplasm). Acute myeloblastic (non-lymphoblastic) leukemia (AML) .. M0 - without cell maturation, myelogenous differentiation is proven 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 whose cells cannot be identified as myeloblastic or lymphoblastic (either by chemical or 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 European American classification of Lymphoid neoplasms), revised (European American) classification of lymphoid hemoblastoses. Pre B cell tumors. Pre B lymphoblastic leukemia/lymphoma. Pre T cell tumors. Pre T lymphoblastic leukemia/lymphoma. Peripheral B cell tumors.. chronic lymphocytic leukemia/small lymphocyte lymphoma.. Lymphoplasmacytic lymphoma.. Mantle cell lymphoma.. Follicular lymphoma.. Marginal zone cell lymphoma.. Hairy cell leukemia.. Plasmacytoma/plasmacytic myeloma.. Diffuse lymphoma of large lymphocytes.. Burkett's lymphoma. Tumors of peripheral T cells and NK cells.. T cell chronic lymphocytic leukemia.. Leukemia of large granular lymphocytes.. Mycosis fungoides and Sézary syndrome T cell lymphoma.. Angioimmunoblastic T cell lymphoma.. Angiocentric lymphoma (NK and T cell lymphoma)... Intestinal T cell lymphoma. Leukemia/adult T cell 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 panmyelosis with myelofibrosis. Acute biphenotypic leukemia with multilineage dysplasia.

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

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

. Acute myeloblastic leukemia occur more often in adults, the subtype depends on the level of cell differentiation. In most cases, the myeloblast clone originates 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 lineages. AML is observed most often; 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 caused by 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 leukemia (M4 and M5, respectively) are characterized by a predominance of non-erythroid cells such as monoblasts. M4 and M5 account for 5-10% of all AML cases. A common symptom is the formation of extramarrow foci of hematopoiesis in the liver, spleen, gums and skin, hyperleukocytosis exceeding 50-100109/l. Sensitivity to therapy and survival rate are lower than with other types of acute myeloblastic leukemia. Erythroleukemia (M6). A variant of acute myeloblastic leukemia, accompanied by increased proliferation of erythroid precursors; characterized by the presence of abnormal blast nucleated red blood cells. The effectiveness of treatment for erythroleukemia is similar to the results of treatment for other subtypes or slightly lower. Megakaryoblastic leukemia (M7) is a rare variant combined with bone marrow fibrosis (acute myelosclerosis). Doesn't respond well to therapy. The prognosis is unfavorable.
Pathogenesis is caused by the proliferation of tumor cells in the bone marrow and their metastasis to various organs. Inhibition of normal hematopoiesis is associated with two main factors: . damage and displacement of the normal hematopoietic lineage by poorly differentiated leukemic cells. production of inhibitors by blast cells that suppress the growth of normal hematopoietic cells.

Stages of acute leukemia. Primary is the active phase. Remission (with treatment) is complete clinical and 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% .. Extramarrow... Neuroleukemia (neurological symptoms, cytosis of more than 10 cells, blasts in the cerebrospinal fluid) ... Testicular (increase in the size of one or two testicles , the presence of blasts is confirmed by cytological and histological studies) .. Mixed. Terminal phase (in the absence of treatment and resistance to therapy)

Symptoms (signs)

Clinical picture of acute leukemia determined by the degree of bone marrow infiltration by blast cells and inhibition of hematopoietic germs. Suppression of bone marrow hematopoiesis.. Anemic syndrome (myelophthisic anemia).. Hemorrhagic syndrome (due to thrombocytopenia, skin hemorrhages are noted - petechiae, ecchymosis; bleeding from the mucous membranes - nosebleeds, internal bleeding).. Infections (impaired function of leukocytes). Lymphoproliferative syndrome.. Hepatosplenomegaly.. Enlarged lymph nodes. Hyperplastic syndrome.. Bone pain.. Lesions of the skin (leukemids), meninges (neuroleukemia) and internal organs. Intoxication syndrome.. Loss of body weight.. 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 accelerated cell life cycle. Hypofibrinogenemia and increased content of fibrin destruction products due to concomitant DIC. The influence of drugs. GC should not be prescribed until a definitive diagnosis has been made. High sensitivity of blast cells to prednisolone leads to their destruction and transformation, making diagnosis difficult.
Treatment is complex; the goal is to achieve complete remission. Currently, hematology centers use various chemotherapy protocols based on the principles of polychemotherapy and intensification of treatment.

. Chemotherapy consists of several stages.. Induction of remission... For ALL - one of the regimens: combinations of vincristine IV weekly, prednisolone orally daily, daunorubicin and asparaginase for 1-2 months continuously... For AML - combination of cytarabine IV drip or subcutaneous injection, intravenous daunorubicin, sometimes in combination with thioguanine. More intensive post-induction chemotherapy, which destroys the remaining leukemic 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 involvement).. Maintenance therapy: periodic courses of remission reinduction.

AML M3 is treated with retinoic acid (tretinoin).
. Bone marrow transplantation is the method of choice for acute myeloblastic leukemia and for relapses of all acute leukemia. 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 surgery, chemotherapy can be administered in ultra-high doses, alone or in combination with radiation therapy (with the goal of completely destroying leukemia cells).. The optimal donor is an identical twin or sibling; More often, donors with a 35% Ag HLA match are used. In the absence of compatible donors, autotransplantation of bone marrow taken during the period of remission is used. The main complication is graft-versus-host disease. It develops as a result of transplantation of donor T-lymphocytes, which recognize the recipient's Ags as foreign and cause an immune reaction against them. An acute reaction develops within 20-100 days after transplantation, a delayed one - after 6-12 months... The main target organs are the skin (dermatitis), the gastrointestinal tract (diarrhea) and the liver (toxic hepatitis)... Treatment is long-term, usually limited prescribing combinations of prednisolone, cyclosporine and small doses of azathioprine. The course of the post-transplantation period is also influenced by preparatory treatment regimens, the development of interstitial pneumonia, and graft rejection (rarely).

. Replacement therapy.. Transfusion of red blood cells to maintain the Hb level not lower than 100 g/l. Transfusion conditions: unrelated donor, use of leukocyte filters. Transfusion of fresh platelets (reduces the risk of bleeding). Indications: platelet count less than 20109/l; hemorrhagic syndrome with platelet content 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 times / day), ventilation and quartzing of rooms; the use of disposable instruments, sterile clothing for medical personnel.. Preventive use of antibiotics, antifungal and antiviral drugs (if the content of segmented neutrophils is less than 0.5109/l, prevention of Pneumocystis pneumonia is indicated)... If the body temperature increases, clinical and bacteriological studies are carried out and immediately begin treatment with combinations of broad-spectrum bactericidal antibiotics: cephalosporins, aminoglycosides and semi-synthetic penicillins... For secondary increases in body temperature that occur after treatment with broad-spectrum antibiotics, antifungal agents (amphotericin B) are empirically used... For the prevention and treatment of neutropenia, colony-stimulating agents can be prescribed factors (for example, molgramity).

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

Age characteristics
. Children.. 80% of all acute leukemias are ALL.. Adverse prognostic factors for ALL... Age of the child under 1 year and over 10 years... Male gender... T - cell variant of ALL... Leukocyte content at the time of diagnosis more 20109/l... Absence of clinical and hematological remission against the background of induction.. Prognosis and course. 80% of clinical and 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 myeloblastic leukemia.

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

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 that lack 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, and exposure to ionizing radiation can cause somatic mutations in hematopoietic tissue. Among mutant pluripotent cells close to the stem cell, a clone may be formed that is insensitive to immunoregulatory influences. From the mutant clone, a tumor consisting of blasts of the same type is formed that intensively proliferates and metastasizes beyond the bone marrow. A distinctive feature of tumor blasts is their 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 profound disorders in all parts of the immune system, and profound dystrophic changes 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) leukemia.

Acute non-lymphoblastic (myeloid) leukemia.

Acute lymphoblastic leukemia is divided into 3 types:

L1 - acute microlymphoblastic type. Antigenic markers of blasts correspond to null (“neither T nor B”) or thymus-dependent (T) lines of lymphopoiesis. Occurs mainly in children.

L2 - acute lymphoblastic. Its substrate is typical lymphoblasts, the antigenic markers of which are the same as in the L1 type of acute leukemia. More common in adults.

L3 - acute macrolymphocytic and prolymphocytic leukemia. Blasts have antigenic markers of B lymphocytes and are morphologically similar to Burkitt lymphoma cells. This type is rare. It has a very poor prognosis.

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

M0 - acute undifferentiated leukemia.

M1 - acute myeloblastic leukemia without cell ripening.

M2 - acute myeloblastic leukemia with signs of cell ripening.

M3 - acute promyelocytic leukemia.

M4 - acute myelomonoblastic leukemia.

M5 - acute monoblastic leukemia.

M6 - acute erythromyelosis.

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”. Body temperature suddenly rises, chills, sore throat, arthralgia, and severe general weakness appear. Less commonly, the disease may first manifest itself as thrombocytopenic purpura, recurrent nasal, uterine, and gastric bleeding. Sometimes acute illness begins with a gradual deterioration of the patient’s condition, the appearance of mild arthralgia, bone pain, and bleeding. In isolated cases, asymptomatic onset of the disease is possible.

In many patients in the initial period of acute illness, enlargement of peripheral lymph nodes and moderate splenomegaly are detected.

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, pain in the left hypochondrium in the area of ​​the spleen, and bleeding. At this stage, clinical syndromes typical of OL are formed:

Hyperplastic (infiltrative) syndrome.

Enlargement of the lymph nodes and spleen is one of the most typical manifestations of dissemination of a leukemic tumor. Leukemic infiltration often causes subcapsular hemorrhages, infarctions, and splenic ruptures.

The liver and kidneys are also enlarged due to leukemic infiltration. Leukemic filtrates in the lungs, pleura, and mediastinal lymph nodes manifest themselves as symptoms of pneumonia and exudative pleurisy.

Leukemic infiltration of the gums with their swelling, hyperemia, and ulcerations is a common occurrence for acute monocytic leukemia.

Localized tumor masses (leukemids) in the skin, eyeballs, and other places occur in non-lymphoblastic (myeloid) forms of leukemia in the later stages of the disease. In some myeloblastic leukemias, the leukemides may have a greenish color (“chloroma”) due to the presence of myeloperoxidase in the tumor blast cells.

Anemic syndrome.

Leukemic infiltration and metabolic inhibition of normal bone marrow hematopoiesis lead to the development of aplastic anemia. Anemia is usually normochromic. 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.

Caused by thrombocytopenia, DIC syndrome. It manifests itself as subcutaneous hemorrhages (thrombocytopenic purpura), bleeding gums, nosebleeds, and uterine bleeding. Gastrointestinal and pulmonary bleeding, gross hematuria are possible. Along with hemorrhages, thrombophlebitis, thromboembolism, and other hypercoagulation disorders caused by disseminated intravascular coagulation syndrome often occur. This is one of the characteristic manifestations of acute promyelocytic and myelomonoblastic leukemia.

Immunodeficiency syndrome.

The formation of an immunodeficiency state is caused by the displacement of normal clones of immunocompetent cells from the bone marrow by leukemic blasts. Clinically manifested by fever, often of the hectic type. Foci of chronic infection of different localization appear. The occurrence of ulcerative necrotizing tonsillitis, peritonsillar abscesses, necrotizing gingivitis, stomatitis, pyoderma, pararectal abscesses, pneumonia, pyelonephritis is typical. Generalization of infection with the development of sepsis, multiple abscesses in the liver, kidneys, hemolytic jaundice, DIC syndrome is often the cause of death of the patient.

Neuroleukemia syndrome.

It is characterized by metastatic spread of foci of blast proliferation into the meninges, brain matter, spinal cord structures, and nerve trunks. Manifested by meningeal symptoms - headache, nausea, vomiting, blurred vision, stiff neck. The formation of large tumor-like leukemic infiltrates in the brain is accompanied by focal symptoms and paralysis of the cranial nerves.

Remission achieved as a result of treatment.

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

Relapse (second and subsequent attacks).

As a result of ongoing mutations, a clone of tumor blasts arises that is able to “evade” the effects of cytostatic drugs used for maintenance treatment. An exacerbation of the disease occurs with the return of all syndromes typical of stages of advanced clinical and hematological manifestations of OA.

Under the influence of anti-relapse therapy, remission can be achieved again. Optimal treatment tactics can lead to recovery. If there is insensitivity to the treatment, OA enters 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 insufficiency or complete absence of therapeutic control over the proliferation and metastasis of the leukemic tumor clone. As a result of diffuse infiltration of the bone marrow and internal organs by leukemic blasts, the normal hematopoietic system is completely suppressed, infectious immunity disappears, and profound disturbances in the hemostatic system occur. Death occurs from disseminated infectious lesions, intractable bleeding, and severe intoxication.

Clinical features of morphological types of acute leukemia.

Acute undifferentiated leukemia (M0). Rarely seen. Progresses very quickly with worsening severe aplastic anemia and severe hemorrhagic syndrome. Remissions are rarely achieved. Average life expectancy is less than 1 year.

Acute myeloblastic leukemia (M1-M2). The most common type of acute non-lymphoblastic leukemia. Adults get sick more often. It is distinguished by a severe, persistently progressive course with pronounced anemic, hemorrhagic, and immunosuppressive syndromes. Ulcerative-necrotic lesions of the skin and mucous membranes are characteristic. 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 severe hemorrhagic syndrome, which most often leads to death of the patient. Violent hemorrhagic manifestations are associated with DIC syndrome, the cause of which is an increase in thromboplastin activity of leukemic promyelocytes. Their surface and cytoplasm contain 10-15 times more thromboplastin than normal cells. Timely treatment allows achieving 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 myeloblastic leukemia. The differences are a greater tendency to necrosis. DIC syndrome occurs more often. Every tenth patient has neuroleukemia. The disease progresses rapidly. Severe infectious complications often occur. The average life expectancy and the frequency of persistent remissions are two times less than for acute myeloblastic leukemia.

Acute monoblastic leukemia (M5). Rare form. Clinical manifestations differ little from myelomonoblastic leukemia. It is characterized by a greater tendency to rapid and persistent progression. Therefore, the average life expectancy of patients with this form of leukemia is even shorter - about 9 months.

Acute erythromyelosis (M6). Rare form. A distinctive feature of this form is persistent, deep anemia. Hyperchromic anemia with symptoms of mild hemolysis. Megaloblastoid abnormalities are detected in leukemic erythroblasts. 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. Accompanied by enlargement of peripheral lymph nodes, spleen, and liver. Hemorrhagic syndrome and ulcerative-necrotic complications are rare. Life expectancy for acute lymphoblastic leukemia is from 1.5 to 3 years.

There are several different treatment options for patients with adult-onset ALL.
  Some treatments are standard (currently used) and some new treatments are being tested in clinical trials. A clinical trial is a research study designed to improve a standard treatment or obtain information about the results of new treatments for cancer patients. If clinical trials show that a new treatment is better than a standard treatment, the new treatment may subsequently become the standard treatment. Patients can also take part in clinical trials. Some clinical trials can only enroll patients who have not received any treatment.
  Treatment for adult acute lymphoblastic leukemia usually occurs in two stages.
  Stages of treatment for adult acute lymphoblastic leukemia:
  Remission-induction therapy. The goal of this stage of treatment is to destroy leukemia cells in the blood and bone marrow and achieve remission.
  Post-remission therapy. This is the second stage of treatment. It begins as soon as remission is achieved. The goal of post-remission therapy is to destroy the remaining leukemia cells, which may not be active, but may subsequently begin to grow and this will lead to relapse. This stage is also called continuation of remission therapy.
  Therapeutic and prophylactic therapy of the central nervous system is usually carried out at each stage of treatment. Because chemotherapy drugs are taken orally or injected intravenously, the drug often fails to destroy leukemia cells in the central nervous system (the brain and spinal cord). Leukemia cells find refuge (hide) in the central nervous system. Intrathecal chemotherapy and radiation therapy can destroy leukemia cells that have entered the central nervous system, thereby preventing relapse of the disease. This type of treatment is called therapeutic and preventive therapy of the central nervous system.
  Today there are four standard treatment methods:
  Chemotherapy.
  Chemotherapy is a method of treating cancer with potent chemotherapy drugs. Chemotherapy drugs can stop and destroy the growth of cancer cells, preventing their separation and penetration into other tissues and organs. During chemotherapy, medications can be taken orally (in the form of tablets, capsules) or given intravenously or intramuscularly. The drug enters the bloodstream, spreads throughout the body and affects cancer cells (systematic chemotherapy). When chemotherapy drugs are injected directly into the spine (intrathecal chemotherapy), organ, or cavity (such as the abdomen), the drug primarily targets cancer cells in those areas (regional chemotherapy). Combination chemotherapy is a treatment that uses more than one cancer chemotherapy drug. The method of using chemotherapy depends on the type and stage of cancer.
  Intrathecal chemotherapy can be used to treat adult ALL, which tends to spread to the brain and spinal cord. Therapy used to prevent cancer cells from spreading in the body and reaching the brain or spinal cord is called CNS treatment. Intrathecal chemotherapy is given in combination with conventional chemotherapy, in which drugs are taken orally or by injection.
  Intrathecal chemotherapy. Antineoplastic agents are injected into the intrathecal cavity of the spinal canal, where the cerebrospinal fluid is located (CSF is shown in blue in the figure). There are two different ways to administer chemotherapy drugs. The first method, shown at the top of the figure, is to administer the drug in the Ommaya reservoir. (A convex container that is inserted into the ventricles of the brain. The container holds the bulk of the drug so that the drug can slowly flow into the brain through small tubes). Another method, shown at the bottom of the figure, injects the drug directly into the cerebrospinal fluid into the spinal column at the lumbar level. The procedure is carried out under local anesthesia.
  Radiation therapy.
  Radiation therapy is a cancer treatment that uses hard X-rays or other types of radiation to kill cancer cells or prevent cancer cells from growing. There are two types of radiation therapy. External beam radiation therapy – a special device focuses radiation into the tumor area. Internal radiation therapy is the use of radioactive substances hermetically sealed in needles, capsules, rods or catheters that are placed directly in or near the tumor. External beam radiation therapy can be used to treat adult ALL, which tends to spread to the brain and spinal cord. This is called therapeutic and preventive therapy of the central nervous system.
  Chemotherapy followed by stem cell transplantation.
  Chemotherapy is given before stem cell transplantation. Stem cell transplantation is used to replace abnormal blood-forming cells with healthy ones. Stem cells (immature blood cells) are taken from the blood or bone marrow of the patient or donor, frozen and stored. At the end of chemotherapy, the stored stem cells are thawed and given to the patient in the form of stem cell infusions. The transplanted stem cells take root and help restore bone marrow cells that produce blood cells.
  Tyrosine kinase inhibitor therapy.
  Anticancer drugs called tyrosine kinase inhibitors are used to treat some types of adult ALL. The drug blocks the enzyme, tyrosine kinase, which promotes the development of large numbers of white blood cells (granulocytes or blast cells) from stem cells. Currently, two such drugs are used: Imatinib (Gleevec) (imatinib mesylate) (Gleevec) and Dasatinib.
  Several new treatments are being tested in clinical trials.
  This section describes treatments that are in clinical trials. It is impossible to talk about all the new treatments that are being studied. Information about clinical trials is available on the NCI website.
  Biological therapy.
  Biological therapy is a treatment that uses the patient's immune system to fight cancer. Substances that are produced in the body or that are synthesized in the laboratory are used to stimulate or restore natural defense mechanisms and fight cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
  Patients can also take part in clinical trials.
  For some patients, participating in a clinical trial is the best choice. Clinical trials are part of the research process. The purpose of clinical trials is to determine whether a new treatment is safe and effective or better than the standard treatment.
  Many of the current standard treatments are based on the results of early clinical trials. Patients participating in clinical trials may receive standard treatment or undergo a new treatment.
  Patients who take part in clinical trials make a major contribution to research and help improve the way cancer is treated in the future. Even if the results of clinical trials do not indicate the effectiveness of a new treatment, they often provide answers to very important questions and help move research one step forward.
  Patients can participate in clinical trials before, during, and after they begin treatment.
  Some clinical trials can only enroll patients who have not received any treatment. Patients whose disease cannot be treated can also participate in clinical trials. There are also clinical trials that explore new ways to prevent recurrence or treat side effects resulting from cancer treatment.
  Carrying out a re-examination.
  Some tests that were done to diagnose cancer or the stage or form of the disease may be repeated. Sometimes tests are repeated to monitor the effectiveness of treatment. The decision to continue, change, or stop treatment is based on the results of these tests.
  Some tests need to be done from time to time and after treatment ends. Test results may show a change in the patient's condition or the presence of a relapse of the disease. Sometimes such tests are called control tests.

Chronic lymphocytic leukemia is an oncological disease in which there is an accumulation of atypical B-lymphocytes in the liver, bone marrow, lymph nodes, spleen and peripheral blood. At the beginning of its development it manifests itself as lymphocytosis, gradually acquiring additional symptoms. Due to decreased immunity, patients often suffer from various types of infections. Increased bleeding and petechial hemorrhages also occur.

Most experts agree that the main cause of the development of lymphocytic leukemia is a hereditary predisposition

The progression of chronic lymphocytic leukemia is accompanied by the following changes in the lymph nodes:

1. In the neck and armpit area, the lymph nodes increase in size.
2. The nodes of the mediastinum and abdominal cavity are affected.
3. The lymph nodes of the groin area are the last to be affected. On palpation, their mobility and high density are noted.

With the development of chronic lymphocytic leukemia, the size of the nodes can reach 5-7 centimeters, representing a noticeable cosmetic defect.

Due to the strong enlargement of the liver, spleen and lymph nodes, nearby organs are subject to compression, which leads to the development of functional disorders in their work.

The main symptoms of chronic lymphocytic leukemia:

• chronic fatigue;
• severe fatigue;
• general decrease in working capacity;
• sleep disturbance;
• dizziness.

When occurring in a latent form, the disease can be detected by chance during a blood test. As a rule, the development of the disease is indicated by an increase in lymphocytes (up to 85-90%). At the same time, red blood cells and platelets have normal levels. In rare cases, patients may develop thrombocytopenia.

In advanced forms of the disease, the following clinical manifestations are possible:

• increased sweating at night;
• weight loss;
• slight rise in temperature.

As a rule, patients experience a decrease in immune tension, leading to the frequent development of urethritis, cystitis, and diseases of a viral and bacterial nature. Even a small wound can fester, and ulcers often appear in the fatty tissue.

Important! It is infectious diseases that can lead to death in lymphocytic leukemia.

Quite often, pneumonia develops against the background of the disease, which causes impaired ventilation of the lungs due to the collapse of lung tissue. Another complication of chronic lymphocytic leukemia is exudative pleurisy, which can lead to compression or rupture of the lymphatic duct located in the chest.

It is not uncommon for patients with lymphocytic leukemia to develop generalized herpes zoster.

More rare complications are:

• hearing loss;
• infiltration of nerve roots, meninges and medulla by lymphocytes;
• the occurrence of tinnitus.

Lymphocytic leukemia may degenerate into Richter syndrome, called diffuse lymphoma. In the latter case, rapid growth of the lymph nodes is observed with the spread of the pathological process to organs not related to the lymphatic system. Only 5-6% of patients survive to develop this stage of lymphocytic leukemia. Death occurs as a result of the development of internal bleeding, anemia, exhaustion of the patient and complications caused by infections. It is also possible to develop acute renal failure due to infiltration of kidney tissue by lymphocytes.

Diagnostics

In case of chronic lymphocytic leukemia, a blood test should be done to identify the number of lymphocytes in the blood, an increase in which leads to the development of the disease

In the vast majority of cases, the disease is discovered completely by accident. As a rule, this happens when undergoing examination for other diseases. In some cases, lymphocytic leukemia is discovered during a routine examination.

It is possible to establish an accurate diagnosis as a result of a comprehensive diagnosis, which includes the following steps:

• listening to patient complaints and taking anamnesis;
• inspection;
• deciphering the blood picture in chronic lymphocytic leukemia and the results of the immunophenotyping procedure.

The main criterion for diagnosing chronic lymphocytic leukemia is an increase in the number of lymphocytes in the blood. Experts also examine the immunophenotype of lymphocytes.

During laboratory diagnostics, it is possible to identify the presence of the following deviations from the norm:

• lymphocytes have increased sizes;
• there are shadows of Gumprecht;
• small B lymphocytes are present;
• Atypical lymphocytes are detected.

The stage of the disease is determined by examining the lymph nodes. To draw up a treatment plan, cytogenetic diagnostics is necessary. If a specialist suspects the development of lymphoma, the patient is referred for a biopsy. It is possible to perform a sternal (from the sternum) bone marrow puncture.

Classification

Taking into account the morphological signs and symptoms of the disease, as well as its rate of development and response to therapy, several forms of this disease are distinguished:

1. Chronic lymphocytic leukemia, characterized by a benign course. With this disease, the patient remains in a satisfactory condition for a long time. The number of leukocytes in the blood is slowly increasing. After diagnosis, lymph nodes can remain normal for decades, and patients lead a normal lifestyle, maintaining their ability to work.
2. Tumor form. It is characterized by a pronounced enlargement of lymph nodes against the background of mild leukocytosis.
3. Classic shape. Also called progressive. Unlike the slowly developing benign form, the symptoms of this pathology increase over several months. In parallel with the deterioration of the patient's condition, an enlargement of the lymph nodes occurs.
4. Bone marrow form. Characterized by progressive cytopenia. Lymph nodes, spleen and liver are not enlarged in chronic lymphocytic leukemia of this type.
5. T cell form. A very rare disease that develops in only 5% of cases. Accompanied by infiltration of the dermis and characterized by rapid progression.
6. Hairy cell leukemia. Lymph nodes are not enlarged, but splenomegaly and cytopenia are detected. Examination of the cellular structure under a microscope shows the presence of characteristic breaks in the cytoplasm of lymphocytes, as well as sprouts like villi at the edges.
7. Lymphocytic leukemia with enlarged spleen. The most striking symptom of pathology is an increase in the size of this organ.
8. Prolymphocytic form. Lymphocytes taken from bone marrow and blood, lymph nodes and spleen contain nucleoli (nucleoli), which mature cells do not normally contain.
9. Lymphocytic leukemia with paraproteinemia. The symptoms are similar to the ailments described above. An additional symptom is monoclonal G- or M-gammopathy.

Depending on the degree of manifestation, three stages of this disease are distinguished:

1. Initial. It has no clinical symptoms and is detected during random diagnosis.
2. Developed clinical manifestations.
3. Terminal. The disease is at an advanced stage and often leads to death.

Features of treatment

The dose and regimen of therapy are determined individually depending on the patient’s condition.

Treatment of chronic lymphocytic leukemia is carried out depending on the stage of the disease and the patient’s health status. For example, if the disease is at an early stage and does not have any manifestations, then doctors choose a wait-and-see approach. It involves undergoing examinations every three months. Treatment is not carried out until the course of the disease begins to worsen. The pathology may not develop for decades.

The reason for prescribing therapy is an increase in the number of leukocytes at least twice over a short period (up to 6 months). In this case, the patient is most often prescribed chemotherapy, which involves a combination of the following drugs:

• Fludarabine;
• Rituximab;
• Cyclophosphamide.

If chronic lymphocytic leukemia continues to progress, the patient is prescribed a large amount of hormonal drugs and preparations for a bone marrow transplant operation begin.

Carrying out chemotherapy and surgery in old age is dangerous. Therefore, such patients are prescribed monochemotherapy (Chlorambucil) or combine this drug with Rituximab.

Forecast

At the moment, there is not a single case of complete recovery from chronic lymphocytic leukemia, but long-term remission is possible.

The survival prognosis depends on a number of factors, including the patient’s age, gender, health status, timely diagnosis of the disease, etc. As a rule, the degree of survival can vary within a very wide range - from a couple of months to several decades.

The disease is characterized by a certain unpredictability. In some cases, patients with a favorable prognosis died from complications of this disease.