Blood transfusion in oncology. The course and clinical forms of acute leukemia, acute leukemia

The task of hemocomponent therapy is the replacement of blood cells depending on the needs of the patient. Whole blood transfusions are used extremely rarely (massive bleeding in the absence of other erythrocyte-containing media).

At planning transfusions of erythrocyte components in patients with acute leukemia, it must be borne in mind that:
1) with leukocytosis more than 100 10 9 /l, transfusions are carried out only after a significant decrease in the number of leukocytes due to the high risk sudden death due to cerebral leukostasis;
2) in patients receiving massive infusion therapy and having increased risk the development of acute left ventricular failure and pulmonary edema, it is necessary to prescribe prophylactic diuretics;
3) with deep thrombocytopenia, transfusion of large volumes of erythromass can lead to an even greater decrease in the number of platelets due to hemodilution (in these cases, platelet transfusion should be performed first).

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There is a direct connection between development of hemorrhagic syndrome and the content of platelets is less than 5-10 10 9 /l. Therefore, platelet transfusions should be performed not only with the development of bleeding, but also for the prevention of hemorrhagic diathesis. In uncomplicated thrombocytopenia, thrombocytopenia transfusions should be performed when the platelet level is less than 20 10 9 /l.

At feverish patients, patients with severe mucositis or coagulopathy require prophylactic platelet transfusions and with a higher platelet count in peripheral blood - more than 20 10 9 /l. The standard dose of platelets is 4-6 units/m 2 per day (1 unit of platelet concentrate contains 50-70 10 9 cells). The exception is patients with promyelocytic leukemia who require massive (up to 20 doses per day) platelet transfusions.

part sick refractoriness to platelet transfusions develops. This may be the result of alloimmunization in patients with multiple transfusions, a consequence of fever, or the development of DIC. The current strategy to overcome alloimmunization includes the prevention of sensitization through the use of related donor or HLA-compatible platelets, as well as the use of leukocyte filters.

At patients who are scheduled for allogeneic myelotransplantation should avoid platelet transfusions from potential bone marrow donors.

Transfusion of fresh frozen plasma (FFP) for coagulopathy in patients with acute leukemia

Table of contents of the topic "Treatment of acute leukemia":

What is this disease?

Acute leukemia is characterized by the accumulation of blast (immature) leukocytes in the bone marrow, lymph nodes, and other organs and tissues. With adequate treatment, some patients (especially children) can live for years and years. So, with lymphoblastic acute leukemia, remission can be achieved in about 90% of children and 65% of adults. Intensive therapy gives the best results in children aged 2 to 8 years.

Acute leukemia is more common in men, residents of industrial areas.

What are the causes of leukemia?

Experts involved in the study of the problem of leukemia, believe that the development of acute leukemia is predisposed to: the combined effect of viruses, genetic and immunological factors, as well as radiation and certain chemicals.

TALK WITHOUT INTERMEDIARIES

Common questions about bone marrow donation

How do I know if I can be a donor?

First of all, fabrics must be compatible. Poorly matched bone marrow can cause a rejection reaction in immune system, and the recipient's condition will become threatening. Before you become a donor, a blood sample will be taken from you, your blood cells will be mixed with the recipient's blood cells and cultured in a special solution. If the cells begin to die, then the tissues are not compatible. If the cells remain healthy, they are compatible and you can be a donor.

Is the procedure for harvesting bone marrow for transplantation dangerous?

The risk of serious complications is very small. There were no deaths or irreversible changes among donors. To protect yourself from blood diseases, you can donate some of your blood to a blood bank in advance. It will come in handy in case of unlikely complications.

How much bone marrow is usually required for a transplant?

Approximately 5% of your bone marrow cells will be taken from you. The body quickly produces bone marrow cells, so in a few weeks its number will be completely restored. Within a few days after the procedure, you will feel completely healthy.

Is it painful?

IN for a day or two you will feel some stiffness and soreness, so you will be prescribed pain medication.

It is assumed that the mechanism of the development of the disease is as follows: at first, immature, non-functioning leukocytes accumulate in the tissues where they originated; then they enter the bloodstream, and from it - into other tissues, disrupting their functioning.

What are the symptoms of the disease?

Usually the disease begins violently. High fever, hemorrhages and bleeding without apparent reason(eg, from the nose, gums), prolonged menstrual bleeding, tiny red or purple spots on the skin.

A few days or weeks before the acute manifestation of the disease, weakness, loss of strength, pallor of the skin, chills and susceptibility to infections develop. In addition, some forms of acute leukemia can lead to shortness of breath, anemia, fatigue, general malaise, palpitations, heart murmurs, stomach pain, and bone pain.

PREVENTIVE MEASURES

How to avoid infection

Follow your doctor's instructions

Take all medicines as directed. Do not stop taking your medications until your doctor tells you to.

Do it all medical appointments so that the doctor can evaluate the changes taking place and the effect of the drugs.

If you need to see another doctor or dentist, be sure to let them know that you are taking an immunosuppressant.

Avoid sources of infection

To minimize the likelihood of infection, avoid crowded places and contact with patients with colds, flu, chickenpox, shingles and other infections.

Do not use any vaccines, especially live ones (for example, against polio), without the permission of a doctor. These vaccines contain weakened but live viruses that can cause illness in those taking immunosuppressant drugs. Also avoid contact with recently vaccinated people.

Check your mouth and skin daily. Keep an eye on them for rashes, cuts, or other lesions.

Wash your hands thoroughly before preparing food, as well as all foods. Make sure they are properly processed.

Learn to recognize danger

Learn to recognize early signs and symptoms of infection: sore throat, chills, feeling tired and lethargic. Seek immediate medical attention if you think you are developing an infectious disease.

Treat minor skin lesions with an antibiotic ointment. In case of deep skin damage, swelling, redness, soreness, consult a doctor immediately.

Follow all the rules of sanitation and hygiene

Monitor the condition of the oral cavity, follow the rules of personal hygiene. If you notice signs of inflammation or sores in your mouth, tell your doctor.

Don't use pre-made mouthwashes because the alcohol and sugar they contain can irritate your mouth and encourage bacteria to grow.

How is the disease diagnosed?

Acute leukemia is diagnosed on the basis of a medical history, physical examination, and analysis of a bone marrow sample that contains a very large number of immature white blood cells. If a particular bone marrow sample does not contain leukemia cells, a bone marrow puncture is performed and the punctate is analyzed.

Indicative blood tests that usually detect low content platelets (blood cells involved in blood clotting). A lumbar puncture is performed to rule out meningitis.

How is acute leukemia treated?

Acute leukemia is treated with chemotherapy, which kills the leukemia cells and leads to remission. The choice of drugs used in chemotherapy depends on the type of acute leukemia.

A bone marrow transplant may be used (see TYPICAL QUESTIONS ABOUT BONE MARROW DONATION). Antibiotics, antifungals and antiviral drugs, as well as injections of granulocytes (a type of white blood cell) to fight infections. Some patients are given platelet transfusions to prevent bleeding, and red blood cell transfusions to prevent anemia.

What should a person with leukemia do?

Watch for signs of infection (fever, chills, cough, sore throat) and signs of bleeding (bruising, small red or purple spots on the skin). Seek medical attention immediately if any are found. stop bleeding pressure bandages or by applying ice to the bleeding site (see HOW TO AVOID INFECTION).

Eat high-calorie foods with lots of protein; Nutrition plays a big role in healing. Be aware that chemotherapy and prednisone can lead to weight gain.

To prevent constipation, drink plenty of fluids, use stool softeners if needed, and go for walks.

If the oral mucosa is inflamed or ulcerated, use a soft toothbrush and avoid hot and spicy foods, and do not use ready-made mouth rinses.

Leukemia is a systemic blood disease and is characterized by some features. First of all, it is progressive cellular hyperplasia in all organs of hematopoiesis, as well as quite often in peripheral blood with the presence of proliferative processes over normal processes hematopoiesis.

Leukemia with metaplastic proliferation of various pathological elements that curl from the original cells and constitute the morphological essence of a particular type of leukemia. Also, the processes occurring in leukemia are called hemoplastoses and are analogous to tumors in other organs. The part that develops directly in the bone marrow is called leukemia. There is also another part that develops directly in lymphoid tissue hematopoietic organs and is called hematosarcomas or lymphomas. There are three groups of diseases, the causes of which may be the following:

• infectious and viral causes;
• hereditary factors of a different plan, which is most often confirmed after long-term observations of a particular family;
• the action of chemical leukemia factors, such as cytostatics, which are necessary for the treatment of cancer, or various antibiotics penicillin series.

To determine the degree of leukemia (acute or chronic) and choose a further treatment strategy, an analysis is performed on the number of blast cells in the blood:

Blood transfusion for leukemia

Transfusion is enough serious procedure, therefore, it is not only not necessary to carry it out in a free manner, but it is also prohibited. Even despite the fact that today quite a lot is treated with transfusion various diseases, some rules must be followed. This is especially true for the selection of the group and the Rh factor of blood.

As for the direct transfusion with serious illnesses, then this procedure can be carried out in different ways. Different blood components can be transfused, depending on what the patient needs. It can be separately plasma, as well as erythrocytes, platelets or leukocytes. For this, a special medical device is used, which separates the blood into separate components.

As for the direct blood transfusion for leukemia, in this case such a procedure is done with a shortage of red blood cells. In the body they play enough important role because they carry oxygen to all tissues. The lack of platelets is also no exception in patients with leukemia. In such cases, a donor is selected for the patient and only what is needed for treatment is taken from the blood. Everything else is poured back to the donor. It is worth saying that the transfusion of such a plan is less dangerous and gentle for a person.

If, however, with the complete selection of blood, the body “gets poorer” a little, then with this method, practically nothing is lost. Returning back the entire blood plasma, all constituent components are quickly restored. Thus, such transfusions can be carried out more often than usual with all the constituent components.

Who can be a donor for a leukemia transfusion?

No matter what kind of transfusion a patient needs, the requirements for donors are the same. Before you donate blood, you need to know with accuracy all your diseases and possible surgeries. This primarily applies to women who have already given birth or during lactation.

It is mandatory to monitor your lifestyle before surrendering two to three days in advance. Drinking alcohol, coffee and other invigorating drinks is not allowed. You must provide a list of all medical preparations that you may have received. This can be one of the causes of blood incompatibility.

Also, do not smoke for 3-4 hours before donating blood. As for the amount of change, it is also determined individually. For example, women are allowed to be a donor no more than once every two months. Only during this time can all components be completely updated. Men can safely donate blood once a month in the amount of no more than 500 ml.

Need for a blood transfusion

In patients with leukemia, the level of platelets and red blood cells is most often significantly reduced due to severe or partial loss of blood. With leukocytosis, there is a significant decrease in blood density, so there are frequent bleeding from the nose. Thus, the necessary normal operation the amount of all components of the blood, and the body begins to suffer.

It can be said that in this disease, transfusion only helps to replenish the state of the erythrocyte and platelet mass for a while. For example, with complex diseases such as lymphoma, leukemia, or myeloma, patients almost always need such a transfusion of donated blood.

In cancerous diseases, the displacement of healthy cells by cancerous ones occurs quite quickly, so patients almost always need a transfusion. If such a procedure is not carried out, then a person’s life can end much earlier, even at the very effective treatment expensive drugs. Also in addition, appropriate chemotherapy is needed, which is also actively involved in the destruction of healthy cells. If all the time only to destroy both cancerous and hematopoietic stem cells, then the result of the treatment will be negative and the person will not survive.

Possible adverse reactions after transfusion

During all this time medical practice there have been quite a few cases where patients complained of adverse reactions after transfusion. This:

• chills and fever;
• various allergic reactions;
• darkening and turbidity of urine;
• pain directly at the site of infusion;
• nausea or vomiting;
• chest pain.

All of the above reactions, as a rule, do not last long and are quite easy to eliminate. But, despite this, some of them can become the most dangerous for the patient. That is why, after a blood transfusion, you need to carefully monitor the patient, monitor his well-being and, if necessary, stop the procedure in time. If the patient begins to feel a slight malaise or nausea during the transfusion, it is necessary to immediately stop the infusion.

Who needs donated blood?

A transfusion is required for every person who suffers from cancer blood. No exception are various general ailments that were provoked by a large loss of blood. For example, this could happen after a complex operation or childbirth in women. In such cases, a simple replacement of all constituent components is required, which will help the body cope with the complication.

As for the immediate complex disease such as leukemia, in this case, transfusion is simply necessary and it is done regularly in order to prolong the life of the patient. This is explained by the fact that treatment alone will not be enough, and chemotherapy generally kills not only diseased cells, but also healthy hematopoietic ones. Without a transfusion, the person will not recover and the treatment will not be effective.

Leukemia -- systemic disease blood, characterized the following features: 1) progressive cellular hyperplasia in the organs of hematopoiesis, and often in peripheral blood with a sharp predominance of proliferative processes over the processes of normal differentiation of blood cells; 2) metaplastic proliferation of various pathological elements developing from the original cells, constituting the morphological essence of a particular type of leukemia.

Diseases of the blood system are hemoblastoses, which are analogous to tumor processes in other organs. Some of them primarily develop in the bone marrow and are called leukemias. And the other part primarily occurs in the lymphoid tissue of the hematopoietic organs and is called lymphomas or hematosarcomas.

Leukemia is a polyetiological disease. Each person may have various factors that caused the disease. There are four groups:

1 group- infectious-viral causes;

2 group- hereditary factors. It is confirmed by observation of leukemia families, where one of the parents is ill with leukemia. According to statistics, there is either direct or one-generation transmission of leukemia.

3 group- the action of chemical leukemia factors: cytostatics in the treatment of cancer lead to leukemia, antibiotics of the penicillin series and cephalosporins. These medicines should not be abused.
Industrial and household chemicals (carpets, linoleum, synthetic detergents, etc.)

4 group- Radiation exposure.

The primary period of leukemia (latent period - the time from the moment of action etiological factor that caused leukemia, before the first signs of the disease. This period can be short (several months) or long (tens of years).
There is a multiplication of leukemia cells, from the first single to such an amount that causes oppression of normal hematopoiesis. Clinical manifestations depend on the rate of reproduction of leukemic cells.

Secondary period (the period of a detailed clinical picture of the disease). The first signs are often detected in the laboratory. There can be two situations:

A) the patient's state of health does not suffer, there are no complaints, but signs (manifestation) of leukemia are noted in the blood;

B) there are complaints, but there are no changes in the cells.

Clinical signs

Leukemia has no characteristic clinical signs, they can be any. Depending on the oppression of hematopoiesis, the symptoms manifest themselves in different ways.

For example, the granulocytic germ (granulocyte - neutrophil) is depressed, one patient will have pneumonia, the other will have tonsillitis, pyelonephritis, meningitis, etc.

All clinical manifestations are divided into 3 groups of syndromes:

1) infectious-toxic syndrome, manifested in the form of various inflammatory processes and is caused by inhibition of the granulocytic germ;

2) hemorrhagic syndrome, manifested by increased bleeding and the possibility of hemorrhages and blood loss;

3) anemic syndrome, manifested by a decrease in the content of hemoglobin, erythrocytes. Appears pallor of the skin, mucous membranes, fatigue, shortness of breath, dizziness, decreased cardiac activity.

Acute leukemia

Acute leukemia is malignant tumor blood systems. The main substrate of the tumor are young, so-called blast cells. Depending on the morphology and cytochemical parameters of cells in the group of acute leukemia, there are: acute myeloblastic leukemia, acute monoblastic leukemia, acute myelomonoblastic leukemia, acute promyelocytic leukemia, acute erythromyelosis, acute megakaryoblastic leukemia, acute undifferentiated leukemia, acute lymphoblastic leukemia.

During acute leukemia, several stages are distinguished:

1) initial;

2) deployed;

3) remission (complete or incomplete);

4) relapse;

5) terminal.

initial stage acute leukemia is diagnosed most often when patients with previous anemia develop a picture of acute leukemia in the future.

Expanded stage characterized by the presence of the main clinical and hematological manifestations of the disease.

Remission may be complete or incomplete. Complete remission includes conditions in which there are no clinical symptoms of the disease, the number of blast cells in the bone marrow does not exceed 5% in the absence of them in the blood. The composition of peripheral blood is close to normal. With incomplete remission, there is a clear clinical and hematological improvement, but the number of blast cells in the bone marrow remains elevated.

relapse acute leukemia can occur in the bone marrow or outside the bone marrow (skin, etc.). Each subsequent relapse is prognostically more dangerous than the previous one.

terminal stage acute leukemia is characterized by resistance to cytostatic therapy, severe inhibition of normal hematopoiesis, development of ulcerative necrotic processes.

IN clinical course of all forms, there are much more common "acute leukemia" features than differences and features, but the differentiation of acute leukemia is important for predicting and choosing cytostatic therapy. Clinical symptoms are very diverse and depend on the localization and massiveness of leukemic infiltration and on signs of suppression of normal hematopoiesis (anemia, granulocytopenia, thrombocytopenia).

The first manifestations of the disease are general character: weakness, loss of appetite, sweating, malaise, abnormal temperature rise, joint pain, slight bruising after minor injuries. The disease can begin acutely - with catarrhal changes in the nasopharynx, tonsillitis. Sometimes acute leukemia is detected by a random blood test.

In the advanced stage of the disease, several syndromes can be distinguished in the clinical picture: anemic syndrome, hemorrhagic syndrome, infectious and ulcerative-necrotic complications.

Anemic syndrome is manifested by weakness, dizziness, pain in the heart, shortness of breath. Objectively marked pallor of the skin and mucous membranes. The severity of anemia is different and is determined by the degree of inhibition of erythropoiesis, the presence of hemolysis, bleeding, and so on.

Hemorrhagic syndrome occurs in almost all patients. Gingival, nasal, uterine bleeding, hemorrhage on the skin and mucous membranes are usually observed. at injection sites and intravenous injections extensive hemorrhages occur. In the terminal stage, at the site of hemorrhages in the mucous membrane of the stomach, intestines, ulcerative necrotic changes. The most pronounced hemorrhagic syndrome is observed in promyelocytic leukemia.

Infectious and ulcerative-necrotic complications are a consequence of granulocytopenia, a decrease in the phagocytic activity of granulocytes and occur in more than half of patients with acute leukemia. Pneumonia, sore throats, infections often occur urinary tract, abscesses at injection sites. The temperature can be different - from subfebrile to constantly high. A significant increase in lymph nodes in adults is rare, in children it is quite common. Lymphadenopathy is especially characteristic for lymphoblastic leukemia. More often, lymph nodes in the supraclavicular and submandibular regions increase. On palpation, the lymph nodes are dense, painless, may be slightly painful with rapid growth. An increase in the liver and spleen is not always observed, mainly with lymphoblastic leukemia.

In the peripheral blood, most patients have normochromic anemia, less often hyperchromic type. Anemia deepens with the progression of the disease up to 20 g/l, and the number of erythrocytes is below 1.0 g/l. Anemia is often the first manifestation of leukemia. The number of reticulocytes is also reduced. The number of leukocytes is usually increased, but does not reach such high numbers as in chronic leukemia. The number of leukocytes varies widely from 0.5 to 50 - 300 g / l.

Forms of acute leukemia with high leukocytosis are prognostically less favorable. Forms of leukemia are observed, which from the very beginning are characterized by leukopenia. Total blast hyperplasia in this case occurs only in the terminal stage of the disease.

For all forms of acute leukemia, a decrease in the number of platelets to 15–30 g/l is characteristic. Especially pronounced thrombocytopenia is observed in the terminal stage.

In the leukocyte formula - blast cells up to 90% of all cells and a small amount mature elements. The release of blast cells into the peripheral blood is the main morphological sign of acute leukemia. To differentiate the forms of leukemia, in addition to morphological features, cytochemical studies are used (lipid content, peroxidase activity, glycogen content, acid phosphatase activity, nonspecific esterase activity, etc.)

Acute promyelocytic leukemia is characterized by an extremely malignant process, a rapid increase in severe intoxication, a pronounced hemorrhagic syndrome, leading to cerebral hemorrhage and death of the patient.

Tumor cells with coarse granularity in the cytoplasm make it difficult to determine the structures of the nucleus. Positive cytochemical signs: peroxidase activity, a lot of lipids and glycogen, a sharply positive reaction to acid phosphatase, the presence of glycosaminoglycan.

Hemorrhagic syndrome depends on severe hypofibrinogenemia and excessive thromboplastin content in leukemic cells. The release of thromboplastin provokes intravascular coagulation.

Acute myeloid leukemia is characterized by a progressive course, severe intoxication and fever, early onset clinical and hematological decompensation of the process in the form of severe anemia, moderate intensity of hemorrhagic manifestations, frequent ulcerative necrotic lesions of the mucous membranes and skin.

Myeloblasts predominate in the peripheral blood and bone marrow. Cytochemical examination reveals peroxidase activity, an increase in lipid content, and low activity of nonspecific esterase.

Acute lymphomooblastic leukemia is a subtype of acute myeloid leukemia. According to the clinical picture, they are almost identical, but the myelomonoblastic form is more malignant, with more pronounced intoxication, profound anemia, thrombocytopenia, more pronounced hemorrhagic syndrome, frequent necrosis of the mucous membranes and skin, hyperplasia of the gums and tonsils. In the blood, blast cells are detected - large, irregular in shape, with a young nucleus, resembling the nucleus of a monocyte in shape. In a cytochemical study in cells, a positive reaction to peroxidase, glycogen and lipids is determined. A characteristic feature is a positive reaction to nonspecific esterase in cells and lysozyme in serum and urine.

The average life expectancy of patients is half that of myeloblastic leukemia. The cause of death is usually infectious complications.

Acute monoblastic leukemia is a rare form of leukemia. The clinical picture resembles acute myeloid leukemia and is characterized by an anemic tendency to hemorrhages, enlarged lymph nodes, enlarged liver, ulcerative necrotic stomatitis. In the peripheral blood - anemia, thrombocytopenia, lymphomonocytic profile, increased leukocytosis. Young blast cells appear. Cytochemical examination in cells is determined by a weakly positive reaction to lipids and high activity nonspecific esterase. Treatment rarely causes clinical and hematological remissions. The life expectancy of the patient is about 8-9 months.

Acute lymphoblastic leukemia is more common in children and young adults. Characterized by an increase in any group of lymph nodes, spleen. The state of health of patients does not suffer, intoxication is expressed moderately, anemia is insignificant. Hemorrhagic syndrome is often absent. Patients complain of pain in the bones. Acute lymphoblastic leukemia differs in frequency neurological manifestations(neuroleukemia).

In the peripheral blood and in the punctate-lymphoblast, young large cells with a rounded nucleus. Cytochemical examination: the reaction to peroxidase is always negative, lipids are absent, glycogen in the form of large granules.

A hallmark of lymphoblastic acute leukemia is a positive response to the therapy used. The frequency of remission -- from 50% to 90%. Remission is achieved by using the complex cytostatic agents. Relapse of the disease can be manifested by neuroleukemia, infiltration of nerve roots, bone marrow tissue. Each subsequent relapse has a worse prognosis and is more malignant than the previous one. In adults, the disease is more severe than in children.

Erythromyelosis is characterized by the fact that the pathological transformation of hematopoiesis concerns both white and red sprouts of the bone marrow. In the bone marrow, young undifferentiated cells of the white row and blast anaplastic cells of the red germ are found - erythro- and normoblasts in large numbers. red cells large sizes have an ugly look.

In the peripheral blood - persistent anemia, anisocytosis of erythrocytes (macrocytes, megalocytes), poikilocytosis, polychromasia and hyperchromia. Erythro- and normoblasts in peripheral blood - up to 200-350 per 100 leukocytes. Leukopenia is often noted, but there may be a moderate increase in leukocytes up to 20–30 g/l. As the disease progresses, blast forms-monoblasts appear. Lymphadenopathy is not observed, the liver and spleen may be enlarged or remain normal. The disease proceeds longer than the myeloid form, in some cases there is a subacute course of erythromyelosis (up to two years without treatment).

The duration of continuous maintenance therapy should be at least 3 years. For early detection of recurrence, it is necessary to perform control studies bone marrow at least 1 time per month in the first year of remission and 1 time in 3 months after a year of remission. During the period of remission, the so-called immunotherapy can be carried out, aimed at destroying the remaining leukemic cells with the help of immunological methods. Immunotherapy consists of administering the BCG vaccine or allogeneic leukemic cells to patients.

Relapse of lymphoblastic leukemia is usually treated with the same combinations of cytostatics as during the induction period.

With non-lymphoblastic leukemia, the main task is usually not to achieve remission, but to contain the leukemic process and prolong the life of the patient. This is due to the fact that non-lymphoblastic leukemias are characterized by a sharp inhibition of normal hematopoietic sprouts, and therefore intensive cytostatic therapy is often impossible.

For the induction of remission in patients with non-lymphoblastic leukemia, combinations of cytostatic drugs are used; cytosine-arabinoside, daunomycin: cytosine - arabinoside, thioguanine; cytosine-arabinoside, oncovin (vincristine), cyclophosphamide, prednisolone. The course of treatment lasts 5-7 days followed by 10-14 afternoon break necessary to restore normal hematopoiesis, inhibited by cytostatics. Maintenance therapy is carried out with the same drugs or their combinations used during the induction period. Almost all patients with non-lymphoblastic leukemia develop a relapse, requiring a change in the combination of cytostatics.

An important place in the treatment of acute leukemia is occupied by the therapy of extramedullary localizations, among which the most frequent and formidable is neuroleukemia (meningo-encephalitic syndrome: nausea, vomiting, unbearable headache; syndrome of local damage to the substance of the brain; pseudotumor focal symptoms; disorder of the functions of the cranial nerves ; oculomotor, auditory, facial and trigeminal nerves; leukemic infiltration of nerve roots and trunks: polyradiculoneuritis syndrome). The method of choice for neuroleukemia is the intralumbar administration of methotrexate and head irradiation at a dose of 2400 rad. In the presence of extramedullary leukemic foci (nasopharynx, testis, mediastinal lymph nodes, etc.), causing compression of organs and pain syndrome, shows local radiation therapy in a total dose of 500--2500 rad.

Treatment infectious complications carried out with broad-spectrum antibiotics directed against the most common pathogens - Pseudomonas aeruginosa, Escherichia coli, Staphylococcus aureus. Apply carbenicillin, gentamicin, tseporin. Antibiotic therapy is continued for at least 5 days. Antibiotics should be given intravenously every 4 hours.

To prevent infectious complications, especially in patients with granulocytopenia, careful care of the skin and oral mucosa, placement of patients in special aseptic wards, intestinal sterilization with non-absorbable antibiotics (kanamycin, rovamycin, neoleptsin) is necessary. The main treatment for hemorrhage in patients with acute leukemia is platelet transfusion. At the same time, the patient is transfused with 200-10,000 g/l of platelets 1-2 times a week. In the absence of platelet mass, fresh whole blood can be transfused for hemostatic purposes or direct transfusion can be used. In some cases, to stop bleeding, the use of heparin (in the presence of intravascular coagulation), epsilon-aminocaproic acid (with increased fibronolysis) is indicated.

Modern programs for the treatment of lymphoblastic leukemia make it possible to obtain complete remissions in 80-90% of cases. The duration of continuous remissions in 50% of patients is 5 years or more. In the remaining 50% of patients, therapy is ineffective and relapses develop. With non-lymphoblastic leukemia, complete remissions are achieved in 50--60% of patients, but relapses develop in all patients. The average life expectancy of patients is 6 months. The main causes of death are infectious complications, severe hemorrhagic syndrome, neuroleukemia.

Chronic myeloid leukemia

The substrate of chronic myeloid leukemia is mainly maturing and mature cells of the granulocytic series (metamyelocytes, stab and segmented granulocytes). The disease is one of the most common in the group of leukemias, occurs rarely in people aged 20-60 years, in the elderly and children and lasts for years.

The clinical picture depends on the stage of the disease.

There are 3 stages of chronic myeloid leukemia - initial, advanced and terminal.

In the initial stage chronic myeloid leukemia is practically not diagnosed or detected by a random blood test, since there are almost no symptoms during this period. Attention is drawn to the constant and unmotivated leukocytosis with a neutrophilic profile, a shift to the left. The spleen is enlarged, which causes discomfort in the left hypochondrium, a feeling of heaviness, especially after eating. Leukocytosis increases to 40--70 g/l. An important hematological sign is an increase in the number of basophils and eosinophils of different maturity. Anemia is not observed during this period. Thrombocytosis up to 600-1500 g/l is noted. In practice, this stage cannot be distinguished. The disease, as a rule, is diagnosed at the stage of total generalization of the tumor in the bone marrow, i.e., in the advanced stage.

Expanded stage characterized by the appearance of clinical signs of the disease associated with the leukemic process. Patients report fatigue, sweating, subfebrile temperature, weight loss. There are heaviness and pain in the left hypochondrium, especially after walking. At objective research an almost constant sign during this period is an enlargement of the spleen, reaching in some cases a significant size. On palpation, the spleen remains painless. Half of the patients develop splenic infarcts, manifested by acute pain in the left hypochondrium with irradiation to the left side, left shoulder, aggravated by deep inspiration.

The liver is also enlarged, but its size is individually variable. Functional disorders liver are expressed slightly. Hepatitis is manifested by dyspeptic disorders, jaundice, enlargement of the liver, increased direct bilirubin in blood. Lymphadenopathy in the advanced stage of chronic myeloid leukemia is rare, hemorrhagic syndrome is absent.

Violations may be observed of cardio-vascular system(pain in the heart, arrhythmia). These changes are due to intoxication of the body, increasing anemia. Anemia has a normochromic character, aniso- and poikilocytosis is often expressed. The leukocyte formula contains the entire granulocytic series, including myeloblasts. The number of leukocytes reaches 250-500 g/l. The duration of this stage without cytostatic therapy is 1.5-2.5 years. The clinical picture changes markedly during treatment. The state of health of patients remains satisfactory for a long time, working capacity is maintained, the number of leukocytes is 10–20 g/l, there is no progressive enlargement of the spleen. The extended stage in patients taking cytostatics lasts 4-5 years, and sometimes more.

In the terminal stage noted sharp deterioration general condition, increased sweating, persistent unmotivated fever. There are severe pains in the bones and joints. An important sign is the appearance of refractoriness to ongoing therapy. Significantly enlarged spleen. Anemia and thrombocytopenia are on the rise. With a moderate increase in the number of leukocytes, the formula is rejuvenated by increasing the percentage of immature cells (promyelocytes, myeloblasts and undifferentiated).

Hemorrhagic syndrome, which was absent in the advanced stage, almost always appears in the terminal period. Tumor process in the terminal stage, it begins to spread beyond the bone marrow: leukemic infiltration of the nerve roots occurs, causing radicular pain, subcutaneous leukemic infiltrates (leukemids) are formed, sarcoma growth is observed in the lymph nodes. Leukemic infiltration on the mucous membranes contributes to the development of hemorrhages in them, followed by necrosis. In the terminal stage, patients are prone to the development of infectious complications, which are often the cause of death.

The differential diagnosis of chronic myeloid leukemia should be carried out primarily with leukemoid reactions of the myeloid type (as a result of the body's response to infection, intoxication, etc.). The blast crisis of chronic myeloid leukemia can give a picture resembling acute leukemia. In this case, anamnestic data, pronounced splenomegaly, and the presence of the Philadelphia chromosome in the bone marrow testify in favor of chronic myeloid leukemia.

The treatment of chronic myeloid leukemia in the advanced and terminal stages has its own differences.

In the advanced stage, therapy is aimed at reducing weight tumor cells and aims to preserve the somatic compensation of patients as long as possible and delay the onset of a blast crisis. The main drugs used in the treatment of chronic myelogenous leukemia: myelosan (mileran, busulfan), myelobromol (dibromomannitol), hexophosphamide, dopan, 6-mercaptopurine, radiation therapy 1500-2000 times.

The patient is recommended to eliminate overloads, stay outdoors as much as possible, quit smoking and drinking alcohol. Recommended meat products, vegetables, fruits. Stay (sunbathing) in the sun is excluded. Thermal, physio- and electrical procedures are contraindicated. In case of a decrease in red blood indicators, hemostimulin, ferroplex are prescribed. Courses of vitamin therapy B1, B2, B6, C, PP.

Contraindications to irradiation are blast crisis, severe anemia, thrombocytopenia.

Upon reaching therapeutic effect switch to maintenance doses. X-ray therapy and cytostatics should be used against the background of weekly blood transfusions of 250 ml of one-group blood and the corresponding Rh accessories.

Treatment in the terminal stage of chronic myeloid leukemia in the presence of blast cells in the peripheral blood is carried out according to the schemes of acute myeloid leukemia. VAMP, CAMP, AVAMP, COAP, a combination of vincristine with prednisolone, cytosar with rubomycin. Therapy is aimed at prolonging the life of the patient, since it is difficult to get remission in this period.

The prognosis of this disease is unfavorable. The average life expectancy is 4.5 years, in some patients it is 10-15 years.

Benign subleukemic myelosis

Benign subleukemic myelosis is an independent nosological form among tumors. hematopoietic system. The tumor substrate consists of mature cells of one, two or all three bone marrow sprouts - granulocytes, platelets, less often erythrocytes. Hyperplasia of myeloid tissue (myelosis) develops in the bone marrow, connective tissue(myelofibrosis), there is a neoplasm of pathological osteoid tissue (osteomyelosclerosis). Growth in the bone marrow fibrous tissue is reactive. Gradually, the development of myelofibrosis leads in the terminal stages of the disease to the replacement of the entire bone marrow with scar connective tissue.

It is diagnosed mainly in old age. For a number of years, patients do not show any complaints. As the disease progresses, weakness, fatigue, sweating, discomfort and heaviness in the abdomen appear, especially after eating. There is reddening of the face, itching, heaviness in the head. The main early symptom is enlargement of the spleen, enlargement of the liver is usually not so pronounced. Hepatosplenomegaly can lead to portal hypertension. A frequent symptom of the disease is pain in the bones, which are observed in all stages of the disease, and sometimes for a long time are its only manifestation. Despite the high content of platelets in the blood, hemorrhagic syndrome is observed, which is explained by the inferiority of platelets, as well as a violation of blood coagulation factors.

In the terminal stage of the disease, fever, exhaustion, anemia increase, pronounced hemorrhagic syndrome, and sarcoma growth in tissues are noted.

Changes in the blood in patients with benign subleukemic myelosis resemble the picture of "subleukemic" chronic myeloid leukemia. Leukocytosis does not reach high numbers and rarely exceeds 50 g / l. In the blood formula - a shift to the left to metamyelocytes and myelocytes, an increase in the number of basophils. Hyperthrombocytosis can reach 1000 g/l or more. At the beginning of the disease, there may be an increase in the number of red blood cells, which later normalizes. The course of the disease can be complicated by hemolytic anemia of autoimmune origin. In the bone marrow, hyperplasia of granulocytic, platelet and erythroid sprouts is observed along with fibrosis and osteomyelosclerosis. In the terminal stage, there may be an increase in blast cells - a blast crisis, which, unlike chronic myeloid leukemia, is rare.

With small changes in the blood, slow growth of the spleen and liver, active treatment is not carried out. Indications for cytostatic therapy are: 1) a significant increase in the number of platelets, leukocytes or erythrocytes in the blood, especially with the development of relevant clinical manifestations (hemorrhages, thrombosis); 2) the predominance of cellular hyperplasia in the bone marrow over the processes of fibrosis; 3) hypersplenism.

In benign subleukemic myelosis, myelosan 2 mg daily or every other day, myelobromol 250 mg 2-3 times a week, imifos 50 mg every other day are used. The course of treatment is carried out for 2-3 weeks under the control of blood counts.

Glucocorticoid hormones are prescribed for insufficiency of hematopoiesis, autoimmune hemolytic crises, hypersplenism.

With significant splenomegaly, irradiation of the spleen in doses of 400-600 rad can be applied. For the treatment of anemic syndrome, anabolic hormones, red blood cell transfusions are used. Physio-, electro-, thermal procedures are contraindicated for patients. The prognosis is generally relatively favorable, patients can live long years and decades to be in a state of compensation.

erythremia

Erythremia (Wakez's disease) true polycythemia) - chronic leukemia, belongs to the group of benign tumors of the blood system. Tumor proliferation of all hematopoietic germs, especially the erythroid germ, is observed, which is accompanied by an increase in the number of red blood cells (in some cases, leukocytes and platelets), hemoglobin mass and viscosity of circulating blood, and an increase in blood coagulation. An increase in the mass of erythrocytes in the bloodstream and vascular depots determines the features of clinical symptoms, the course and complications of the disease.

Erythremia occurs predominantly in the elderly. There are 3 stages of the course of the disease: initial, deployed (erythremic) and terminal.

In the initial stage, patients usually complain of heaviness in the head, tinnitus, dizziness, fatigue, decreased mental performance, chilliness of extremities, sleep disturbance. External characteristic signs may be absent.

The expanded stage is characterized by more vivid clinical symptoms. The most frequent and hallmark are headaches, which sometimes have the character of excruciating migraines with visual impairment.

Many patients complain of pain in the region of the heart, sometimes such as angina pectoris, pain in the bones, in the epigastric region, weight loss, impaired vision and hearing, unstable mood, tearfulness. A common symptom of erythremia is pruritus. There may be paroxysmal pain in the tips of the fingers and toes. Pain is accompanied by reddening of the skin.

On examination, the typical red-cyanotic color of the skin with a predominance of a dark cherry tone attracts attention. There is also redness of the mucous membranes (conjunctiva, tongue, soft palate). Due to frequent thrombosis of the limbs, darkening of the skin of the legs is observed, sometimes - trophic ulcers. Many patients complain of bleeding gums, bleeding after tooth extraction, bruising on the skin. In 80% of patients, there is an increase in the spleen: in the advanced stage, it is moderately enlarged, in the terminal stage, severe splenomegaly is often observed. The liver is usually enlarged. Often in patients with erythremia, an increase in blood pressure. Hypertension in erythremia is characterized by more pronounced cerebral symptoms. As a result of a violation of the trophism of the mucous membrane and vascular thrombosis, ulcers of the duodenum and stomach may occur. An important place in the clinical picture of the disease is occupied by vascular thrombosis. Thrombosis of the cerebral and coronary arteries, as well as vessels of the lower extremities, is usually observed. Along with thrombosis, patients with erythremia are prone to the development of hemorrhages.

In the terminal stage, the clinical picture is determined by the outcome of the disease - cirrhosis of the liver, coronary thrombosis, a focus of softening in the brain due to thrombosis of the cerebral vessels and hemorrhages, myelofibrosis accompanied by anemia, chronic myeloid leukemia and acute leukemia.

In the peripheral blood in the initial stage of the disease, only moderate erythrocytosis can be observed. A characteristic hematological sign of the advanced stage of erythremia is an increase in the number of erythrocytes, leukocytes, and platelets in the blood (pancytosis). The most typical for erythremia is an increase in the number of erythrocytes up to 6-7 g/l and hemoglobin up to 180-220 g/l. In parallel with an increase in erythrocytes and hemoglobin, an increase in hematocrit is noted.

An increase in the dense part of the blood and its viscosity leads to sharp decline ESR up to the complete absence of erythrocyte sedimentation. The number of leukocytes increased slightly - up to 15-18 g/l. The formula reveals neutrophilia with a stab shift, less often metamyelocytes and myelocytes appear. The number of thrombocytes is increased to 1000 g/l.

Albuminuria is constantly found, sometimes hematuria. In the terminal stage, the blood picture depends on the outcome of erythremia. During the transition to myelofibrosis or myeloid leukemia, the number of leukocytes increases, shift to the left, normocytes appear, the number of erythrocytes decreases. In the case of acute leukemia, blast cells are detected in the blood, anemia and thrombocytopenia are constantly encountered.

In the bone marrow of patients with an advanced stage of erythremia, a typical sign is hyperplasia of all 3 sprouts (panmyelosis) with severe megakaryocytosis. In the terminal stage, myelofibrosis is observed with persistent megakaryocytosis. The main difficulties lie in the differential diagnosis of erythremia with secondary symptomatic erythrocytosis. There are absolute and relative erythrocytosis. Absolute erythrocytoses are characterized increased activity erythropoiesis and an increase in the mass of circulating red blood cells. With relative erythrocytosis, there is a decrease in plasma volume and a relative predominance of erythrocytes per unit volume of blood. The mass of circulating erythrocytes with relative erythrocytosis is not changed.

Absolute erythrocytosis occurs in hypoxic conditions (lung diseases, birth defects heart, altitude sickness), tumors (hypernephroma, adrenal tumors, hepatoma), some kidney diseases (polycystic, hydronephrosis).

Relative erythrocytosis occurs mainly in pathological conditions associated with increased fluid loss (prolonged vomiting, diarrhea, burns, excessive sweating).

In the initial stages of the disease, occurring without pronounced pancytosis, bloodletting of 300–600 ml is indicated 1–3 times a month.
The bleeding effect is unstable. With systematic bloodletting, iron deficiency may develop. In the advanced stage of erythremia in the presence of pancytosis, the development of thrombotic complications, cytostatic therapy is indicated. The most effective cytostatic drug in the treatment of erythremia is imifos. The drug is administered intramuscularly or intravenously at a dose of 50 mg daily for the first 3 days, and then every other day. For the course of treatment - 400-600 mg. The effect of imifos is determined after 1.5-2 months, since the drug acts at the level of the bone marrow. In some cases, anemia develops, which usually gradually disappears on its own. With an overdose of imiphos, hematopoietic hypoplasia may occur, for the treatment of which prednisolone, nerobol, vitamin B6 and B12, as well as blood transfusions are used. The average duration of remission is 2 years, maintenance therapy is not required. With a recurrence of the disease, sensitivity to imiphos persists. With increasing leukocytosis, rapid growth of the spleen, myelobromol is prescribed at a dose of 250 mg for 15-20 days. Less effective in the treatment of erythremia myelosan. Anticoagulants are used as symptomatic treatments for erythremia. antihypertensive drugs, aspirin.

The prognosis is relatively favorable. The total duration of the disease in most cases is 10-15 years, and in some patients it reaches 20 years. Significantly worsen the prognosis vascular complications, which can cause death, as well as the transformation of the disease into myelofibrosis or acute leukemia.

Chronic lymphocytic leukemia

Chronic lymphocytic leukemia is a benign tumor disease of the lymphoid (immunocompetent) tissue, which, unlike other forms of leukemia, does not show tumor progression throughout the course of the disease. The main morphological substrate of the tumor is mature lymphocytes, which proliferate and accumulate in increased numbers in the lymph nodes, spleen, liver, and bone marrow. Among all leukemias, chronic lymphocytic leukemia occupies a special place. Despite the morphological maturity of lymphocytes, they are functionally inferior, which is expressed in a decrease in immunoglobulins. The defeat of the immunocompetent system determines the tendency of patients to infections and the development autoimmune anemia, thrombocytopenia, less often - granulocytopenia. The disease occurs mainly in the elderly, more often in men, often occurs in blood relatives.

The disease begins gradually without severe clinical symptoms. Often the diagnosis is made for the first time with a random blood test, an increase in the number of leukocytes, the presence of lymphocytosis is detected. Weakness gradually appears fatigue, sweating, weight loss. There is an increase in peripheral lymph nodes, mainly in the cervical, axillary and groin areas. Subsequently, the mediastinal and retroperitoneal lymph nodes are affected. On palpation are determined peripheral lymph nodes soft or doughy consistency, not soldered to each other and the skin, painless. The spleen is significantly enlarged, dense, painless. The liver is most often enlarged. On the part of the gastrointestinal tract, diarrhea is noted.

There is no hemorrhagic syndrome in a typical uncomplicated form. Skin lesions are much more common than in other forms of leukemia. Skin changes may be specific or non-specific. Nonspecific include eczema, erythroderma, psoriatic rashes, pemphigus.

Specific ones are leukemic infiltration of the papillary and subpapillary dermis. Skin infiltration can be focal or generalized.

One of clinical features chronic lymphocytic leukemia is the reduced resistance of patients to bacterial infections. Among the most frequent infectious complications are pneumonia, urinary tract infections, tonsillitis, abscesses, septic condition.

A severe complication of the disease is autoimmune processes associated with the appearance of antibodies against antigens of one's own blood cells. The most common is autoimmune hemolytic anemia.

Clinically, this process is manifested by a deterioration in the general condition, an increase in body temperature, the appearance of mild jaundice, and a decrease in hemoglobin. Autoimmune thrombocytopenia accompanied by hemorrhagic syndrome may be observed. Less common is autoimmune lysis of leukocytes.

Chronic lymphocytic leukemia can transform into hematosarcoma - the gradual transformation of enlarged lymph nodes into a dense tumor, severe pain, a sharp deterioration in the general condition.

There are several forms of chronic lymphocytic leukemia:

1) a typical benign form of the disease with a generalized enlargement of the lymph nodes, moderate hepatosplenomegaly, leukemic blood picture, absence of anemia, rare infectious and autoimmune disorders. This form occurs most often and is characterized by a long and favorable course;

2) a malignant variant that differs severe course, the presence of dense lymph nodes that form conglomerates, high leukocytosis, inhibition of normal hematopoiesis, frequent infectious complications;

3) splenomegalic form, often occurring without peripheral lymphadenopathies, often with an increase in abdominal lymph nodes. The number of leukocytes is within the normal range or slightly reduced. Rapidly growing anemia is characteristic;

4) bone marrow form with an isolated lesion of the bone marrow, a leukemic blood picture, and the absence of enlarged lymph nodes and spleen. Often develops anemia, thrombocytopenia with hemorrhagic syndrome;

5) the cutaneous form (Cesari's syndrome) proceeds with predominant leukemic infiltration of the skin;

6) forms with an isolated increase in individual groups of lymph nodes and the presence of appropriate clinical symptoms.

Changes in the peripheral blood are characterized by high leukocytosis up to 20-50 and 100 g/l. Sometimes the number of leukocytes is slightly increased. Lymphocytes make up 60-90% of all shaped elements. The bulk is mature lymphocytes, 5--10% are prolymphocytes. Characteristic of chronic lymphocytic leukemia is the presence of a large number of dilapidated nuclei of lymphocytes with remnants of nucleolus - the "shadow" of Botkin-Gumprecht.

In case of transformation of chronic lymphocytic leukemia into hemosarcoma, lymphocytosis is replaced by neutrophilia.

In the myelogram of patients with chronic lymphocytic leukemia, a sharp increase in the percentage of mature lymphocytes is revealed up to complete metaplasia of the bone marrow by lymphocytes.

In the blood serum, there is a decrease in the content of gamma globulins.

In chronic lymphocytic leukemia, cytostatic and radiation therapy are performed to reduce the mass of leukemia cells. Symptomatic treatment, aimed at combating infectious and autoimmune complications, includes antibiotics, gamma globulin, antibacterial immune sera, steroid drugs, anabolic hormones, blood transfusions, splenectomy.

If you feel unwell with a benign form, a course of vitamin therapy is recommended: B6, B12, ascorbic acid.

With a progressive increase in the number of leukocytes and the size of the lymph nodes, primary restraining therapy is prescribed with the most convenient cytostatic drug chlorbutin (leukeran) in tablets of 2-5 mg 1-3 times a day.

When signs of decompensation of the process appear, cyclophosphamide (endoxan) is most effective intravenously or intramuscularly at the rate of 200 mg per day, for a course of treatment of 6-8 g.

With low efficiency of polychemotherapeutic programs, radiation therapy is used on the area of ​​enlarged lymph nodes and spleen, the total dose is 3000 rad.

In most cases, treatment of chronic lymphocytic leukemia is carried out on an outpatient basis throughout the entire period of the disease, with the exception of infectious and autoimmune complications that require hospital treatment.

The life expectancy of patients with a benign form is an average of 5--9 years. Some patients live 25-30 years or more.

All patients with leukemia are recommended a rational regime of work and rest, nutrition with a high content of animal protein (up to 120 g), vitamins and fat restriction (up to 40 g). The diet should contain fresh vegetables, fruits, berries, fresh herbs.

Almost all leukemias are accompanied by anemia, so herbal medicine rich in iron and ascorbic acid is recommended.

Use an infusion of wild rose hips and wild strawberries for 1/4-1/2 cup 2 times a day. A decoction of wild strawberry leaves is taken 1 glass a day.

Periwinkle pink is recommended, the herb contains more than 60 alkaloids. Of greatest interest are vinblastine, vincristine, leurosine, rosidine. Vinblastine (Rozevin) is effective drug to maintain remissions caused by chemotherapeutic agents. It is well tolerated by patients during long-term (2-3 years) maintenance therapy.

Vinblastine has some advantages over other cytostatics: it has more quick action(this is especially noticeable with high leukocytosis in patients with leukemia), does not have a pronounced inhibitory effect on erythropoiesis and thrombocytopoiesis. That allows sometimes to use it even with mild anemia and thrombocytopenia. It is characteristic that the inhibition of leukopoiesis caused by vinblastine is most often reversible and, with an appropriate dose reduction, can be restored within a week.

Rosevin is used for generalized forms of lymphogranulomatosis, lympho- and reticulosarcoma, chronic myelosis, especially with resistance to other chemotherapeutic drugs and radiation therapy. Enter intravenously 1 time per week, at a dose of 0.025--0.1 mg/kg.

use vitamin tea: rowan fruits - 25 g; rose hips - 25 g. Take 1 glass a day. Infusion of rose hips - 25 g, black currant berries - 25 g. Take 1/2 cup 3-4 times a day.

Apricot fruits contain a large amount of ascorbic acid, vitamins B, P, provitamin A. The fruits contain iron, silver, etc. 100 g of apricot affects the blood formation process in the same way as 40 mg of iron or 250 mg of fresh liver, which determines medicinal value of these fruits for people suffering from anemia.

American avocado, fruits are used in fresh and subjected to various processing. Salads, seasonings are prepared from the fruits, they are used as butter for sandwiches. Taken for the treatment and prevention of anemia.

Common cherry, used in raw, dried and canned form (jam, compotes). Cherry improves appetite, it is recommended as a tonic for anemia. Consume in the form of syrup, tincture, liqueur, wine, fruit water.

Beetroot, cooked various dishes, use it in dried, salted, pickled and canned form. The combination of a large amount of vitamins with iron has a stimulating effect on hematopoiesis.

Blackcurrant, the main advantage of the fruit is the low content of enzymes that destroy ascorbic acid therefore they serve as a valuable source of vitamins. Recommended for hypochromic anemia.

Sweet cherries, fruits can be frozen and dried, compotes, preserves, jams are prepared from it. Effective in hypochromic anemia.

Mulberry is eaten in the form of syrups, compotes, dessert dishes and liqueurs. Used for hypochromic anemia.

Garden spinach, leaves contain proteins, sugars, ascorbic acid, vitamins B1, B2, P, K, E, D2, folic acid, carotene, mineral salts (iron, magnesium, potassium, phosphorus, sodium, calcium, iodine). Leaves are used for food, from which salads, mashed potatoes, sauces, and other dishes are prepared. Spinach leaves are especially useful for patients with hypochromic anemia.

In the diet of patients with anemia include vegetables, berries and fruits as carriers of "factors" of hematopoiesis. Iron and its salts contain potatoes, pumpkin, swede, onion, garlic, lettuce, dill, buckwheat, gooseberries, strawberries, grapes.

Potato, white cabbage, eggplant, zucchini, melon, pumpkin, onion, garlic, rosehip, sea buckthorn, blackberry, strawberry, viburnum, cranberry, hawthorn, gooseberry, lemon, orange, apricot, cherry, pear, ascorbic acid and B vitamins contain corn, etc.

You can use various medicinal plants including the following:

1. Collect buckwheat flowers and prepare an infusion: 1 cup per 1 liter of boiling water. Drink without restriction.

2. Prepare the collection: spotted orchid, two-leaved love, medicinal sweet clover, sowing buckwheat color - all 4 tbsp. l., lobed nightshade, field horsetail - 2 tbsp. l. For 2 liters of boiling water, take 6 tbsp. l. collection, take the first portion of 200 g in the morning, and then 100 g 6 times a day.

3. Collection: medicinal sweet clover, field horsetail, stinging nettle - all 3 tbsp. l. For 1 liter of boiling water, take 4-5 tbsp. l. collection. Take 100 g 4 times a day.

4. Drink juice from mallow roots, and children - juice from mallow fruits.

Acute leukemia (acute leukemia) is a relatively rare variant of the leukemic process with the growth of undifferentiated mother cells instead of normally maturing granular leukocytes, erythrocytes and platelets; clinically manifested by necrosis and septic complications due to the loss of the phagocytic function of leukocytes, severe uncontrollably progressive anemia, severe hemorrhagic diathesis, inevitably leading to death. In its rapid course, acute leukemias are clinically similar to cancers and sarcomas from poorly differentiated cells in young people.

In the development of acute leukemia, one cannot help but see the extreme disorganization of the functions that regulate normal body hematopoiesis, as well as the activity of a number of other systems (defeat vasculature, skin, mucous membranes, nervous system in acute leukemia). In most cases, acute leukemias are acute myeloblastic forms.

Epidemiology of acute blood leukemia

The incidence of acute leukemia is 4-7 cases per 100,000 population per year. An increase in the incidence is observed after 40 years with a peak of 60-65 years. In children (peak 10 years), 80-90% of acute leukemias are lymphoid.

Causes of acute blood leukemia

contribute to the development of the disease viral infections, ionizing radiation. Acute leukemia can develop under the influence of chemical mutants. These substances include benzene, cytostatics, immunosuppressants, chloramphenicol, etc.

Influenced harmful factors changes occur in the structure of the hematopoietic cell. The cell mutates, and then the development of the already changed cell begins, followed by its cloning, first in the bone marrow, then in the blood.

An increase in the number of altered leukocytes in the blood is accompanied by their release from the bone marrow, and then their settlement in various bodies and body systems, followed by dystrophic changes in them.

The differentiation of normal cells is disturbed, this is accompanied by inhibition of hematopoiesis.

The cause of acute leukemia in most cases cannot be determined. The following are some of the congenital and acquired diseases that contribute to the development of leukemia:

• Down syndrome;
• Fanconi anemia;
• Bloom's syndrome;
• Klinefelter's syndrome;
• neurofibromatosis;
• ataxia-telangiectasia.

In identical twins, the risk of acute leukemia is 3-5 times higher than in the general population.

to leukemia factors external environment include ionizing radiation, including exposure to prenatal period, various chemical carcinogens, especially benzene derivatives, smoking (2-fold increased risk), chemotherapy drugs and various infectious agents. Apparently, at least in some cases in children, a genetic predisposition appears in the prenatal period. In the future, after birth, under the influence of the first infections, other genetic mutations may also occur, which eventually becomes the cause of the development of acute lymphoblastic leukemia in children.

Acute leukemia develops as a result of malignant transformation of hematopoietic stem cells or early progenitor cells. Leukemic progenitor cells proliferate without undergoing further differentiation, which leads to the accumulation of power cells in the bone marrow and inhibition of marrow hematopoiesis.

Acute leukemia is caused by chromosomal mutations. They occur under the influence of ionizing radiation, which has shown a 30-50 times increase in the incidence in Hiroshima and Nagasaki. Radiation therapy increases the risk of disease. Cigarette smoke causes at least 20% of acute leukemias. Have carcinogenic effects chemical compounds(benzene, cytostatics). In patients with genetic diseases leukemias are more common. There is evidence that viruses are able to integrate into the human genome, increasing the risk of developing tumors. In particular, the human T-lymphotropic retrovirus causes adult T-cell lymphoma.

Pathological changes concern mainly lymph nodes, lymphatic tissue of the pharynx and tonsils, bone marrow.

Lymph nodes present a picture of metaplasia in character, usually myeloblastic tissue. Necrotic changes predominate in the tonsils. Bone marrow is red, consists mainly of myeloblasts or hemocytoblasts, less often of other forms. Normoblasts and megakaryocytes are found only with difficulty.

The pathogenesis consists in a more rapid growth of a clone of pathological blast cells, which displace the cells of normal hematopoiesis. Leukemia cells can develop on any initial stage hematopoiesis.

Symptoms and signs of acute leukemia, acute leukemia

Acute leukemia is characterized by the following syndromes:

• intoxication;
• anemic;
• hemorrhagic (ecchymosis, petechiae, bleeding);
• hyperplastic (ossalgia, lymphadenopathy, hepatosplenomegaly, gum infiltration, neuroleukemia);
• infectious complications (local and generalized infections).

Acute promyelocytic leukemia is more aggressive and is characterized by a fulminant course. In 90% of patients with acute promyelocytic syndrome, DIC develops.

Acute leukemia is manifested by signs of impaired bone marrow hematopoiesis.

• Anemia.
• Throbocytopenia and associated bleeding.
• Infections (mainly bacterial and fungal).

There may also be signs of extramedullary leukemic infiltration, more often occurring in acute lymphoblastic leukemia and the monocytic form of acute lyeloid leukemia.

• Hepatosplenomegaly.
• Lymphadenopathy.
• Leukemic meningitis.
• Leukemic infiltration of the testicles.
• Skin nodules.

Acute promyelocytic leukemia is manifested by bleeding associated with primary fibrinolysis and disseminated intravascular coagulation (DIC).

Persons of any age get sick, often young.

The doctor sees in front of him a serious patient in a state of prostration, complaining of weakness, shortness of breath, headache, tinnitus, local phenomena in the mouth, pharynx, acutely developed along with a sudden fever and chills, night sweats, vomiting, diarrhea. Patients amaze with extreme pallor, developing from the first days of the disease; large hemorrhages on the skin at the injection site of bone pressure, etc.

Swelling and hyperemia of the mucous membrane of the mouth and nasopharynx, ulcerative necrotic stomatitis, sometimes the nature of a noma, with salivation, fetid breath, an ulcerative necrotic process in the tonsils, spreading to the arches, the back wall of the pharynx, the larynx and leading to perforation of the sky, etc. etc., swelling of the neck with swelling of the lipatic nodes of the anterior cervical triangle.

Less commonly, necrosis affects the vulva and various other organs. There are epistaxis, bloody vomiting due to the collapse of the leukemic infiltrate of the stomach wall, thrombopenia, damage to the vascular wall - a ulcerative necrotic form of acute leukemia, often mistaken for diphtheria or scurbut.

In other cases, necrosis does not develop. Anemia, fever, lack of air when talking and the slightest movements come to the fore, a sharp noise in the head and ears, a puffy face, tachycardia, chills with abnormal temperature rises, hemorrhages in the bottom of the eye, in the brain - an anemic-septic form of acute leukemia, miscible with primary diseases red blood or with sepsis as the underlying disease.

Enlargement of the lymph nodes and spleen in acute leukemia does not reach any significant degree and is often established for the first time only with a systematic study of the patient; sternum, ribs sensitive to pressure due to leukemic growths. On the face usual signs severe anemia - dancing of the arteries, the noise of a top on the neck, systolic murmur on the heart.

Blood changes not limited to leukocytes. They constantly find severe anemia progressive every day with a color index of about one and with a drop in hemoglobin to 20%, and erythrocytes to 1,000,000. The plates sharply decrease in number or completely disappear.

Nuclear erythrocytes are absent, reticulocytes are less than normal, despite severe anemia, anisocytosis and poikilocytosis are not expressed. Thus, red blood is indistinguishable from aplastic anemia-aleukia. The white blood cell count can be normal and even low (why the disease is often not recognized correctly) or increased to 40,000-50,000, rarely more significantly. Characteristically, up to 95-98% of all leukocytes are undifferentiated cells: myeloblasts are usually small, rarely medium and large (acute myeloblastic leukemia); apparently, there may also be acute lymphoblastic forms, or the main representative is an even less differentiated cell of the hemocytoblast character (acute hemocytoblastosis).

There is no difference between these forms. practical value in view of an equally hopeless prognosis; at the same time, it is often difficult even for an experienced hematologist (Myeloblasts are characterized by basophilic protoplasm and a finely reticulated nucleus with 4-5 clearly translucent nucleoli.). The pathologist, formulating final diagnosis, is often based only on the totality of all changes in organs at autopsy. Acute leukemia is characterized by a gap (the so-called hiatus leucaemicus-leukemic gap.) between the dying, non-replenishing mature forms of neutrophils and other leukocytes and the maternal forms, incapable of further differentiation, the absence of intermediate forms, so typical of chronic myeloid leukemia.

The same mechanism explains the irrepressible drop in the number of erythrocytes - mother cells (hemocytoblasts) lose their ability to differentiate and in the direction of erythrocytes in acute leukemia, and mature peripheral blood erythrocytes that are present at the onset of the disease die off in the usual time (about 1-2 months). There is no reproduction and megakaryocytes - hence the sharp thrombopenia, the absence of clot retraction, a positive tourniquet symptom and other provocative phenomena of hemorrhagic diathesis. Urine often contains red blood cells as well as protein.

The disease proceeds in several stages. Available initial stage, advanced stage and stage of remission of the disease.

Body temperature may rise to very high values, acute inflammatory changes in the nasopharynx, ulcerative necrotic tonsillitis appear.

In the advanced stage, all manifestations of the disease are intensified. In the blood, the number of normal clones of leukocytes decreases, the number of mutated cells increases. This is accompanied by a decrease in the phagocytic activity of leukocytes.

Lymph nodes quickly increase in size. They become dense, painful.

In the terminal stage, the general condition deteriorates sharply.

A sharp increase in anemia, a decrease in the number of platelets - platelets, and manifestations of the inferiority of the vascular wall are intensified. There are hemorrhages, bruises.

The course of the disease is malignant.

The course and clinical forms of acute leukemia, acute leukemia

Acute leukemia sometimes develops through one or another period after childbirth, scarlet fever, diphtheria, acute attacks malaria, etc., but no direct connection with any septic or other infection can be established. The disease ends in death after 2-4 weeks (with ulcerative necrotic form) or after 2 or more months (with anemic septic variant); some fluctuations and temporary stops in the progression of the process and a more protracted course of the disease (subacute leukemia) are possible.

Due to the defenselessness of the body due to the almost complete disappearance of mature phagocytic neutrophils, acute leukemia, like agranulocytosis and aleukia, often leads to secondary sepsis with the detection of streptococcus or other pathogens in the blood (sepsis e neutropenia - sepsis due to neutropenia). The immediate cause of death may be pneumonia, blood loss, cerebral hemorrhage, endocarditis.

A peculiar variant of acute or subacute, usually myeloblastic, leukemias are periosteal forms with damage to the skull (and often protrusion of the eye-exophthalmos) and other bones with characteristic green leukemic infiltrates (chlorleukemia, "green cancer").

Prognosis of acute leukemia

Survival of patients who do not receive treatment is usually 3-6 months. The prognosis also depends on a number of factors, such as karyotype, response to therapy, and the general condition of the patient.

Diagnosis and differential diagnosis of acute leukemia, acute leukemia

Most common symptom acute leukemia - pancytopenia, but in a small proportion of patients, the number of leukocytes in the blood is increased.

The diagnosis is made on the basis of a morphological examination of the bone marrow. It allows you to differentiate myeloid leukemia from lymphoid and judge the prognosis of the disease. The diagnosis of acute leukemia is made when the number of power cells is more than 20% of nucleated cells. Leukemic infiltration of the brain tissue is one of the manifestations of acute lymphoblastic leukemia, for its diagnosis it is necessary to study the cerebrospinal fluid.

As mentioned above, acute leukemia is often misdiagnosed as scurvy, diphtheria, sepsis, malaria, with which, however, it has only a superficial resemblance. Agranulocytosis is characterized by a normal number of erythrocytes and plates; hemorrhagic diathesis is absent. With aplastic anemia (aleukia) - leukopenia with a predominance of normal lymphocytes; myeloblasts and other maternal cells are not found in the blood, nor are they found in the bone marrow.

With infectious mononucleosis (glandular fever, Filatov-Pfeifer disease), the number of leukocytes is increased to 20,000-30,000 with an abundance of lympho- and monoblasts, part of the atypical (leukemoid blood picture), in the presence of cyclic fever, tonsillitis, more often of the catarrhal type or with films, swelling of the lymph nodes in the neck, to a lesser extent in other places, enlarged spleen. The general condition of patients suffers a little; red blood remains normal. Usually, recovery occurs in 2-3 weeks, although the lymph nodes may remain enlarged for months. Blood serum agglutinates sheep erythrocytes (Paul-Bunnel reaction).

With an exacerbation of chronic myeloid leukemia, the number of myeloblasts rarely exceeds half of all leukocytes; the total number of leukocytes is often in the hundreds of thousands. Sharply enlarged spleen and lymph nodes. The anamnesis gives indications of a protracted course of the disease.

Differential diagnosis of acute pancytopenia is carried out with diseases such as aplastic anemia, Infectious mononucleosis. In some cases, a high number of blasts may be a manifestation of a leukemoid reaction to an infectious disease (eg, tuberculosis).

Histochemical studies, cytogenetics, immunophenotyping, and molecular biological studies make it possible to differentiate power cells in ALL, AML, and other diseases. For exact definition variant of acute leukemia, which is extremely important when choosing treatment tactics, it is necessary to determine B-cell, T-cell and myeloid antigens, as well as flow cytometry.

In patients with CNS symptoms, head CT is performed. Radiography is performed to determine the presence of a tumor formation in the mediastinum, especially before anesthesia. CT, MRI, or ultrasound can diagnose splenomegaly.

Differential Diagnosis

Differentiate acute leukemia with leukemoid reactions in infectious diseases such as monocytosis in tuberculosis.

And also the disease should be distinguished from lymphomas, chronic leukemia with blast crisis, multiple myeloma.

Treatment of acute leukemia, acute leukemia

• Chemotherapy,
• Supportive care.

The goal of treatment is complete remission, incl. resolution of clinical symptoms, restoration of normal blood cell levels and normal hematopoiesis with the level of power cells in the bone marrow<5% и элиминация лейкозного клона. Хотя основные принципы лечения ОЛЛ и ОМЛ сходны, режимы лечения отличаются. Разнообразие встречающихся клинических ситуаций и вариантов лечения требует участия опытных специалистов. Предпочтительно проведение лечения, особенно его наиболее сложных фаз (например, индукция ремиссии) в медицинских центрах.

Of the cytostatics, mercaptopurine, methotrexate, vincristine, cyclophosphamide, cytosine-arabinoside, rubomycin, krasnitin (L-asparase) are used.

Supportive care. Supportive care for acute leukemia is similar and may include:

• blood transfusion;
• antibiotics and antifungals;
• hydration and alkalization of urine;
• psychological support;

Transfusions of platelets, erythrocytes and granulocytes are performed according to indications in patients with bleeding, anemia and neutropenia, respectively. Prophylactic platelet transfusion is performed at the level of peripheral blood platelets<10 000/мкл; при наличии лихорадки, диссеминированного внутрисосудистого свертывания и мукозита, обусловленного химиотерапией, используется более высокий пороговый уровень. При анемии (Нb <8 г/дл) применяется трансфузия эритроцитартой массы. Трансфузия гранулоцитов может применяться у больных с нейтропенией и развитием грамнегативных и других серьезных инфекций, но ее эффективность в качестве профилактики не была доказана.

Antibiotics are often needed because patients develop neutropenia and immunosuppression, which can lead to rapid infections. After performing the necessary examinations and cultures in patients with fever and neutrophil levels<500/мкл следует начинать лечение антибактериальными препаратами, воздействующими и на грампозитивные и на грамнегативные микроорганизмы.

Hydration (2-fold increase in daily fluid intake), alkalinization of urine, and monitoring of electrolytes can prevent the development of hyperuricemia, hyperphosphatemia, hypocalcemia, and hyperkalemia (tumorlysis syndrome), which are caused by rapid lysis of tumor cells during induction therapy (especially in ALL). Prevention of hyperuricemia is carried out by the appointment of allopurinol or rasburicase (recombinant urate oxidase) before starting chemotherapy.

Treatment until recent years did not make it possible to significantly alleviate the course of the disease. X-ray therapy worsens the course of the disease and is therefore contraindicated.

The treatment of acute leukemia with penicillin proposed in recent years in combination with a transfusion of erythrocyte mass (Kryukov, Vlados) has a beneficial effect on individual manifestations of the disease, often eliminating fever, promoting the healing of necrotic-ulcerative lesions and improving the composition of red blood, and causes a temporary stop in some patients (remission) of the disease. Whole blood transfusion is also recommended. Remission was also obtained from the use of 4-aminopteroylglutamic acid, which is a biological folic acid antagonist; on this basis, it seems necessary to limit the use of other hematopoietic stimulants that accelerate the reproduction of poorly differentiated blood cells. Careful patient care, good nutrition, symptomatic treatment, and remedies that calm the nervous system are necessary.

In the event of an exacerbation of acute leukemia, maintenance therapy is interrupted and replaced by treatment.

Acute lymphoblastic leukemia

Acute lymphoblastic leukemia is the most common type of leukemia in children. It accounts for 23% of malignant neoplasms diagnosed in children under 15 years of age.

Treatment of acute lymphoblastic leukemia

It is important to treat patients with acute lymphoblastic leukemia in specialized centers. There is an increasing understanding that the treatment of adolescents with leukemia is more effective if they are among their peers, which serves as additional support for them.

Treatment of children with leukemia is currently carried out according to the risk group, this approach is increasingly being used in the treatment of adults. The prognostically significant clinical and laboratory signs in children include the following.

• Age at which leukemia was diagnosed. In children younger than 1 year old, the prognosis is unfavorable, in children from 1 to 9 years old, the prognosis is better than in adolescents aged 10-18 years.
• The number of leukocytes in the blood at the time of diagnosis. When the number of leukocytes is less than 50x108 / l, the prognosis is better than when there are more leukocytes.
• Leukemic infiltration of the tissue of the brain or spinal cord is an unfavorable prognostic sign.
• Gender of the patient. Girls have a slightly better prognosis than boys.
• Hypodiploidity (less than 45 chromosomes) of leukemic cells on karyotyping is associated with a worse prognosis than normal chromosomes or hyperdiploidy.
• Specific acquired genetic mutations, including the Philadelphia chromosome t(9;22), and rearrangement of the MLL gene on chromosome 11q23 are associated with poor prognosis. Rearrangement of the MLL gene is often found in acute lymphoblastic leukemia in infants.
• response to therapy. If the child's power cells disappear from the bone marrow within 1 to 2 weeks of starting therapy, the prognosis is better. The rapid disappearance of power cells from the blood under the influence of glucocorticoid therapy is also a favorable prognostic sign.
• The absence of minimal residual disease on molecular studies or flowcytometry indicates a favorable prognosis.

Chemotherapy

Treatment of patients with B-cell acute lymphoblastic leukemia (Burkitt's leukemia) is usually the same as for Burkitt's lymphoma. It consists of short courses of intensive chemotherapy. Patients with the Philadelphia chromosome receive a stem cell transplant and are prescribed imatinib. Treatment takes place in three stages - induction of remission, intensification (consolidation) and maintenance therapy.

remission induction

Remission induction is achieved by the combined administration of vincristine, glucocorticoids (prednisolone or dexamethasone) and asparaginase. Anthracycline is also prescribed for adult patients and high-risk children. Remission occurs in 90-95% of children and a slightly smaller proportion of adults.

Intensification (consolidation)

This is a very important step during which new chemotherapeutic agents (eg, cyclophosphamide, thioguanine, and cytosine arabinoside) are prescribed. These drugs are effective in leukemic infiltration of the brain and spinal cord. CNS lesions can also be treated with radiation therapy and intrathecal or intravenous (in moderate or high doses) methotrexate.

In high-risk patients, the probability of recurrence in the central nervous system is 10%, in addition, various complications are possible in the long-term period.

Supportive care

After achieving remission, patients are treated with methotrexate, thioguanine, vincristine, prednisolone for 2 years, as well as prophylactic intrathecal administration of these drugs, if radiation therapy has not been performed.

Several approaches have been developed for the treatment of patients classified as high-risk category 1. The appointment of large doses of cyclophosphamide or methotrexate in the stage of intensification (consolidation) allows to achieve some success, stem cell transplantation after reaching the first remission leads to recovery of 50% (with allogeneic transplantation) and 30% (with autogenous transplantation) of patients. However, the accumulated experience is insufficient for a comparative evaluation of this method with intensive conventional chemotherapy. If the treatment does not give the desired result, the outcome depends on the age and duration of the first remission. In children with long-term remission, the appointment of chemotherapy often leads to recovery, in other cases, stem cell transplantation is indicated.

Early results in the treatment of patients with the Philadelphia chromosome with the additional appointment of imatinib (Glivec) are very encouraging.

Acute myeloid leukemia

In clinical practice, the following three factors are of great importance for the diagnosis of acute myeloid leukemia and the choice of optimal treatment.

• It is important to recognize acute promyelocytic leukemia, since the inclusion of tretinoin (the full trans isomer of retinoic acid) in the treatment regimen depends on this.
• The age of the patient.
• General condition (functional activity) of the patient. It has now become common practice to intensively treat patients under 60 years of age. Older individuals make up the majority of patients with acute myeloid leukemia and are often not suitable for intensive chemotherapy, so they are limited to palliative treatment with blood products.

Chemotherapy

Anteacycline and cytosine arabinoside prescribed for 7-10 days have been the mainstay of treatment for patients with acute myeloid leukemia for 30 years. A regimen with the addition of thioguanine or etoposide as a third drug is widely used, but data on which regimen is better is not enough. Recently, interest has increased in the appointment of cytosine arabinoside for the induction of remission, there are no convincing data on the advantage of this approach.

Induction is considered successful if the first remission is achieved (normal hemogram and the number of power cells in the bone marrow is less than 5%). It also depends on the age of the patient: remission is achieved in 90% of children, 75% of patients aged 50-60 years, 65% of patients aged 60-70 years. Three to four intensive courses of other drugs, such as amsacrine, etoposide, idarubicin, mitoxantrone, and higher doses of cytosine arabinoside, are also usually given. At present, it remains unclear what number of consolidation rates should be considered optimal. Elderly patients rarely tolerate more than two courses.

Prognostic factors

Based on a number of factors, it is possible to assess the risk of recurrence of the disease, and therefore the patient's chances of survival. The most significant of these factors are cytogenetic (may have a favorable, intermediate, or unfavorable prognostic value), age of the patient (the prognosis is less favorable in older patients), and the primary response of bone marrow power cells to treatment.

Other factors for poor prognosis include the following:

• molecular markers, in particular, internal tandem duplication of the FLT3 gene (detected in 30% of cases, it can predict the recurrence of the disease);
• low degree of differentiation (undifferentiated leukemia);
• leukemia associated with previous chemotherapy:
• the duration of the first remission (remission lasting less than 6-12 months is a sign of an unfavorable prognosis).

Favorable cytogenetic factors include translocations and inversion of inv, which are more often observed in young patients. Unfavorable cytogenetic factors include abnormalities of chromosomes 5, 7, long arm of chromosome 3 or combined anomalies, more often detected in elderly patients with acute myeloid leukemia associated with previous chemotherapy or myelodysplasia. Cytogenetic changes classified as moderate risk include changes that are not included in the two categories described. The phenotype characterized by overexpression of the Pgp glycoprotein, which causes resistance to chemotherapy drugs, is especially often found in elderly patients, it is the cause of a lower remission rate and a high relapse rate in them.

stem cell transplant

Patients younger than 60 years of age can be offered allogeneic stem cell transplantation if there is an HLA-matched donor. For low-risk patients, stem cell transplantation is performed only if first-line therapy is ineffective, and in other cases it is performed as a consolidation. It is difficult to judge the positive effect of stem cell allotransplantation associated with the graft-versus-tumor reaction due to the toxic effect of drugs, although toxic manifestations can be reduced with the use of more gentle pre-transplant preparation regimens. In patients younger than 40 years old, allotransplantation of stem cells is performed after myeloablation, achieved by high-dose chemotherapy in combination with radiation therapy or without it, while in older patients, pre-transplantation preparation is carried out in a more gentle mode, providing only myelosuppression.

Acute promyelocytic leukemia

Treatment with tretinoin (the full trans isomer of retinoic acid) induces remission without causing hypoplasia, but chemotherapy is also needed to destroy the leukemic cell clone, given simultaneously with tretinoin or immediately after completion of treatment with it. An important prognostic factor is the number of leukocytes in the blood at the time of diagnosis. If it is less than 10x106/l, combined therapy with tretinoin and chemotherapy drugs allows 80% of patients to be cured. If the number of leukocytes in the blood exceeds this figure, then 25% of patients are doomed to early death and only 60% have a chance to survive. However, the question of how intensive chemotherapy should be has not been definitively resolved, especially when it comes to treating low-risk patients. In a Spanish study, good results were achieved with treatment with tretinoin in combination with the anthracycline derivative idarubicin (no cytosine arabinoside) followed by maintenance therapy. However, according to a recent European study, anthracyclines and cytosine arabinoside reduced the risk of relapse to a greater extent than anthracycline alone. Patients who have achieved remission are taken under observation, their treatment is resumed when molecular genetic signs of relapse are detected, without waiting for the clinical manifestations of the disease. A new drug for the treatment of recurrences has been developed - arsenic trioxide, which promotes the differentiation of tumor cells.

The results of the treatment of acute myeloid leukemia

Survival depends on the age of the patients and the prognostic factors discussed earlier. Currently, approximately 40-50% of patients younger than 60 years of age survive for a long time after treatment, while only 10-15% of patients older than 60 years of age survive the 3-year milestone. Consequently, in most patients, leukemia recurs. If the first remission is short (3-12 months) and the results of cytogenetic studies are unfavorable, the prognosis is usually poor.

prospects

Acute myelogenous leukemia is a heterogeneous group of diseases, apparently, the treatment of its constituent nosological units requires a separate risk assessment. Thus, the effectiveness of arsenic preparations in acute promyelocytic leukemia has been shown. Currently, work continues to improve the method of treating patients with stem cell transplantation. Immunological methods of treatment will be used more and more widely. Thus, a new anti-SOPZ drug, calicheomycin mylotarg, has already been patented and is being used for the treatment of elderly patients with leukemia. The problem of treating elderly patients is still far from being solved.

Standard chemotherapy regimens have proved to be ineffective, and the 5-year survival rate is approximately 10%. It should be clarified in which cases intensive chemotherapy is justified. To this end, an AML16 study is currently underway in the UK. It is intended to provide a platform for the rapid evaluation of a number of new drugs in phase II randomized trials. These drugs include nucleoside analogues such as clofarabine, inhibitors of FLT3 tyrosine kinase, farnesyl transferase, and histone deacetylase.