Diseases of the hematopoietic system. Blood diseases - classification, signs and symptoms, syndromes of blood diseases, diagnosis (blood tests), methods of treatment and prevention Damage to all blood cells

Diseases of the hematopoietic system are a number of pathologies that entail disturbances in the structure and functional purpose of blood cells: red blood cells, leukocytes, platelets or plasma. Blood cells may begin to be produced in insufficient quantities or in excess, and the plasma may change due to the ingress of pathological protein structures into it.

Today science has studied more than 100 blood diseases. Hematologists treat patients with such ailments.

Causes of blood diseases

1. Hereditary, caused by genetic abnormalities or congenital defects. Here, disturbances at the chromosomal level have a lifelong effect on the number of formed elements in the blood and its overall composition.
2. Acquired due to dietary deficiencies or drug abuse. Factors that provoke these conditions can be radiation, acute poisoning with heavy metals or alcohol surrogates.

Types of blood diseases

Numerous pathologies of hematopoiesis are conventionally combined into 3 large groups:

• anemic, the main characteristic of these diseases is a low level of hemoglobin in the blood;
• diathesis of hemorrhagic nature, when blood clotting time is disrupted;
• tumor processes that can develop in various hematopoietic organs and the circulatory system.

There are also idiopathic diseases of unknown etiology.

Anemia is caused by a decrease in the amount of hemoglobin. Its decrease in the blood can be caused by a violation of its synthesis (insufficient production) or a pathology in the structure of red blood cells, which deliver it to all systems of the body. Also, the pathology can be caused by the accelerated breakdown of hemoglobin or red blood cells, in which case they do not have time to perform their functions.

A separate group of anemias are hemorrhagic diseases associated with blood loss. If a person simultaneously loses half a liter of blood, this is a dangerous acute condition that requires immediate medical attention. Prolonged bleeding of small volumes is classified as chronic; they are not so dangerous, but without appropriate treatment they lead to exhaustion of the body.

Pathologies associated with impaired hemoglobin production are often congenital in nature, but can also occur against the background of poor nutrition, with a decrease in meat dishes in the diet, deficiency of microelements (iron, copper, zinc) or B vitamins, folic acid.

Oncological blood diseases are characterized by blood pathologies at the cellular level; they are divided into leukemia (bone marrow diseases) and lymphosis (diseases of the lymphatic system). If the bone marrow is affected, immature atypical cells begin to multiply in it. Oncology of lymphoid vessels is characterized by disturbances in the cellular structure and the formation of pathological nodes and compactions.

Hemorrhagic diseases are caused by low blood clotting due to a decrease in the number of platelets, these include thrombopenia, disseminated intravascular coagulation syndrome, and vasculitis.

The classification of blood diseases includes diseases that cannot be classified into any of the groups. These are agranulocytosis (deficiency of eosinophils, basophils and neutrophils), eosinophilia - increased production of eosinophils, cytostatic disease - a disease associated with treatment with antitumor drugs.

Symptoms

There are a lot of diseases of the hematopoietic system, and clinical manifestations depend on which blood elements are involved in the process. However, a number of common symptoms can be identified:

• asthenia, decreased performance, weakness, drowsiness;
• rapid heartbeat, frequent dizziness, fainting;
• disturbances of appetite, a feeling of disgust for previously favorite foods and smells or, conversely, an addiction to inhaling toxic substances and consuming inedible foods and substances;
• changes in thermoregulation, chronic fever;
• allergic and skin manifestations;
• high susceptibility to viral and bacterial infections;
• tendency to bleed;
• pain in the liver and spleen, bones and muscles.

Common diseases of the blood and hematopoietic organs

A common blood pathology of hereditary origin is hemophilia. The disease is detected in early childhood and is transmitted through the male line. Due to a defect in the chromosomes, the hematopoietic system is not able to control the bleeding that has begun, so with this disease, blood loss can be very extensive.

Leukemia is a common systemic blood disease. This is an oncological disease that can occur in both acute and chronic forms. With leukemia, the bone marrow is affected and begins to produce abnormal cells instead of healthy ones. The disease is characterized by fever, bone pain, severe asthenic syndrome, and oral pathologies (stomatitis, gingivitis, tonsillitis).

Thrombocytopenia is a common autoimmune blood pathology. Important structural elements of blood, platelets, have defects in their structure, because of this, in the clinic of these diseases, the tendency to external and internal bleeding, headaches and joint pain, and damage to internal organs prevails.

Diagnostics

The presence of blood pathologies is confirmed using laboratory tests; advanced tests with counting of formed elements make it possible to quickly detect disorders in the hematopoietic system. If necessary, blood is tested for fibrinogen or blood clotting rate.

In case of bone marrow diseases, according to the doctor’s indications, a biopsy sample is taken using a puncture. To determine the cause of hereditary diseases, genetic studies are prescribed.

Treatment

Therapy of blood diseases requires an accurate diagnosis; only in this case can everything be cured or the pathology corrected. The general principles of symptomatic treatment include infusion therapy (to relieve fever and intoxication), blood or plasma transfusion (to replenish blood elements), supportive and restorative therapy. Chemotherapy and bone marrow transplantation are used to treat blood tumors.

Blood diseases are dangerous, widespread, the most severe of them are generally incurable and lead to death. Why is such an important system of the body as the circulatory system subject to pathologies? The reasons are very different, sometimes even beyond the control of the person, but accompanying him from birth.

Blood diseases

Blood diseases are numerous and varied in their origin. They are associated with pathology of the structure of blood cells or a violation of the functions they perform. Also, some diseases affect plasma - the liquid component in which cells are located. Blood diseases, a list, and the causes of their occurrence are carefully studied by doctors and scientists, some of which have not yet been determined.

Blood cells - red blood cells, white blood cells and platelets. Erythrocytes - red blood cells - carry oxygen to the tissues of internal organs. Leukocytes - white blood cells - fight infections and foreign bodies that enter the body. Platelets are colorless cells responsible for clotting. Plasma is a viscous protein liquid that contains blood cells. Due to the serious functionality of the circulatory system, blood diseases are mostly dangerous and even difficult to cure.

Classification of diseases of the circulatory system

Blood diseases, the list of which is quite large, can be divided into groups according to their area of ​​distribution:

• Anemia. A condition of pathologically low levels of hemoglobin (this is the oxygen-carrying component of red blood cells).
• Hemorrhagic diathesis - a clotting disorder.
• Hemoblastosis (oncology associated with damage to blood cells, lymph nodes or bone marrow).
• Other diseases that do not belong to the three above.

This classification is general; it divides diseases according to the principle of which cells are affected by pathological processes. Each group contains numerous blood diseases, a list of which is included in the International Classification of Diseases.

List of diseases affecting the blood

If you list all the blood diseases, the list will be huge. They differ in the reasons for their appearance in the body, the specifics of cell damage, symptoms and many other factors. Anemia is the most common pathology affecting red blood cells. Signs of anemia are a decrease in the number of red blood cells and hemoglobin. The reason for this may be reduced production or large blood loss. Hemoblastoses - most of this group of diseases is occupied by leukemia, or leukemia - blood cancer. As the disease progresses, blood cells transform into malignant tumors. The cause of the disease has not yet been clarified. Lymphoma is also an oncological disease; pathological processes occur in the lymphatic system, and leukocytes become malignant.

Myeloma is a blood cancer that affects the plasma. Hemorrhagic syndromes of this disease are associated with a clotting problem. They are mostly congenital, such as hemophilia. It manifests itself as hemorrhages in joints, muscles and internal organs. Agammaglobulinemia is a hereditary deficiency of serum plasma proteins. There are so-called systemic blood diseases, the list of which includes pathologies affecting individual systems of the body (immune, lymphatic) or the entire body as a whole.

Anemia

Let's look at the blood diseases associated with erythrocyte pathology (list). The most common types:

• Thalassemia is a disorder of the rate of hemoglobin formation.
• Autoimmune hemolytic anemia - develops as a result of a viral infection, syphilis. Drug-induced non-autoimmune hemolytic anemia - due to poisoning with alcohol, snake venom, and toxic substances.
• Iron deficiency anemia - occurs when there is a lack of iron in the body or with chronic blood loss.
• B12 deficiency anemia. The reason is a lack of vitamin B12 due to insufficient dietary intake or impaired absorption. The result is a disturbance in the central nervous system and gastrointestinal tract.
• Folate deficiency anemia - occurs due to a lack of folic acid.
• Sickle cell anemia - red blood cells are shaped like a sickle, which is a serious hereditary pathology. The result is slow blood flow, jaundice.
• Idiopathic aplastic anemia is the absence of tissue that reproduces blood cells. Possible due to irradiation.
• Familial erythrocytosis is a hereditary disease characterized by an increase in the number of red blood cells.

Diseases of the hemoblastosis group

These are mainly oncological diseases of the blood; the list of the most common includes types of leukemia. The latter, in turn, are divided into two types - acute (a large number of cancer cells, do not perform functions) and chronic (proceeds slowly, the functions of blood cells are performed).

Acute myeloblastic leukemia is a disorder in the division of bone marrow cells and their maturation. Depending on the nature of the disease, the following types of acute leukemia are distinguished:

• without ripening;
• with maturation;
• promyelocytic;
• myelomonoblastic;
• monoblastic;
• erythroblastic;
• megakaryoblastic;
• lymphoblastic T-cell;
• lymphoblastic B-cell;
• panmyeloid leukemia.

Chronic forms of leukemia:

• myeloid leukemia;
• erythromyelosis;
• monocytic leukemia;
• megakaryocytic leukemia.

The above chronic diseases are taken into account.

Letterer-Siwe disease is the proliferation of immune system cells in various organs; the origin of the disease is unknown.

Myelodysplastic syndrome is a group of diseases affecting the bone marrow, which include, for example,

Hemorrhagic syndromes

• Disseminated intravascular coagulation (DIC) is an acquired disease characterized by the formation of blood clots.
• Hemorrhagic disease of the newborn is a congenital deficiency of clotting factor due to vitamin K deficiency.
• Deficiency of substances that are found in the blood plasma, mainly these include proteins that ensure blood clotting. There are 13 types.
• Idiopathic Characterized by discoloration of the skin due to internal bleeding. Associated with low platelet levels in the blood.

Damage to all blood cells

• Hemophagocytic lymphohistiocytosis. A rare genetic disease. Caused by the destruction of blood cells by lymphocytes and macrophages. The pathological process occurs in various organs and tissues, resulting in damage to the skin, lungs, liver, spleen, and brain.
• caused by infection.
• Cytostatic disease. It manifests itself as the death of cells that are in the process of dividing.
• Hypoplastic anemia is a decrease in the number of all blood cells. Associated with cell death in the bone marrow.

Infectious diseases

Blood diseases can be caused by infections that enter the body. What are the types of infectious blood diseases? List of the most common:

• Malaria. Infection occurs during a mosquito bite. Microorganisms that penetrate the body infect red blood cells, which are destroyed as a result, thereby causing damage to internal organs, fever, and chills. Usually found in the tropics.
• Sepsis - this term is used to refer to pathological processes in the blood, which are caused by the penetration of bacteria into the blood in large quantities. Sepsis occurs as a result of many diseases - diabetes, chronic diseases, diseases of internal organs, injuries and wounds. The best defense against sepsis is good immunity.

Symptoms

Typical symptoms of blood diseases are fatigue, shortness of breath, dizziness, loss of appetite, tachycardia. With anemia due to bleeding, dizziness, severe weakness, nausea, and fainting occur. If we talk about infectious blood diseases, the list of their symptoms is as follows: fever, chills, itchy skin, loss of appetite. With a long course of the disease, weight loss is observed. Sometimes there are cases of perverted taste and smell, as with B12 deficiency anemia, for example. There may be pain in the bones when pressed (with leukemia), enlarged lymph nodes, pain in the right or left hypochondrium (liver or spleen). In some cases, a skin rash and nosebleeds occur. In the early stages, blood diseases may not show any symptoms.

Treatment

Blood diseases develop very quickly, so treatment must begin immediately after diagnosis. Each disease has its own specific characteristics, so treatment is prescribed in each case differently. Treatment of oncological diseases such as leukemia is based on chemotherapy. Other methods of treatment are blood transfusions, reducing the effects of intoxication. In the treatment of blood cancers, stem cell transplants obtained from bone marrow or blood are used. This newest way to fight the disease helps restore the immune system and, if not overcome the disease, then at least prolong the patient’s life. If tests make it possible to determine which infectious blood diseases the patient has, the list of procedures is aimed primarily at eliminating the pathogen. This is where antibiotics come to the rescue.

Causes

There are numerous blood diseases, the list is long. The reasons for their occurrence are different. For example, diseases associated with blood clotting problems are usually hereditary. They are diagnosed in young children. All blood, the list of which includes malaria, syphilis and other diseases, is transmitted through the carrier of the infection. This could be an insect or another person, a sexual partner. such as leukemia, have an unclear etiology. The cause of blood disease can also be irradiation, radioactive or toxic poisoning. Anemia can occur due to poor nutrition, which does not provide the body with the necessary elements and vitamins.

Anemia is a condition characterized by a decrease in the concentration of red blood cells, a decrease in the hemoglobin content per unit volume of blood and the average hemoglobin content in one red blood cell.

In older children, anemia can develop as a result of blood loss, due to helminthiasis and as a manifestation of diseases of other systems and organs (leukemia, nephritis, diffuse liver damage, infections, intoxications, hereditary diseases, etc.). In young children, anemia in 90% of all cases is of a nutritional-infectious nature, i.e., of mixed etiology. These anemias are most often detected in the second half of the year or in the second year of life; Infectious nutritional anemia more often develops at the age of O -18 months. This is explained by the functional lability and slight vulnerability of the hematopoietic apparatus of the infant.

In modern conditions, the most common cause of nutritional-infectious anemia in young children is iron deficiency, which is why these anemias are called iron deficiency. Typically, the disease develops with one-sided, most often milk feeding, with untimely, irrational administration of vitamins, complementary foods, with frequent illnesses, with violation of the regime, errors in care, etc.

The disease develops gradually. The leading symptom is pallor of the skin and visible mucous membranes of varying severity. Often, pallor of the skin is combined with puffiness of the face, posture of the lower extremities, and sometimes edema develops. As a rule, children with anemia easily get cold hands and feet. Constant symptoms of anemia in young children include a sharp decrease, absence or perversion of appetite. Long-term loss of appetite or lack of it leads to slow weight gain.

Quite often, in patients with anemia, an enlarged liver is found, less often - an enlarged spleen. Changes in the central nervous system in the form of lethargy, unmotivated irritability, tearfulness, apathy, and negativism are observed with the same frequency.

Disorders of the cardiovascular system are manifested by tachycardia, the appearance of functional systolic murmur in the cardiac region and vessels of the neck (“spinning top murmur”).

More rare manifestations of anemia include dry skin, roughness, atrophy of the papillae of the tongue, dull hair (sometimes it falls out, becomes thin, brittle), changes in the shape of nails (spoon-shaped), polyadenia, and low-grade fever.

The main changes in the blood are a decrease in the total amount of hemoglobin and a decrease in the concentration of hemoglobin in red blood cells. Most often, the hemoglobin level is 40-100 g/l (4-10 g%). In most patients, the number of red blood cells is moderately reduced and only in rare cases can it be less than 3-10/l. However, it should be borne in mind that with anemia in children, the number of red blood cells may correspond to the average age indicators.

The color index of blood in patients decreases (0.4-0.6), and in some of them significantly (0.3-0.4).

Changes in white blood in most patients are reduced to a moderate increase in the number of leukocytes with the appearance of young forms (band, young and even myelocytes). There may be mild thrombocytopenia.

A characteristic sign of iron deficiency anemia in young children is a decrease in serum iron levels. Compared with the indicators of healthy children (800-1100 mcg/l), the iron content decreases by 2-3 times, and sometimes more, and the degree of its decrease is more significant in children in the first months of life.

Anemia in children occurs differently; there are mild and severe forms.

Mild forms of anemia are characterized by the fact that the child’s general condition remains satisfactory, but at the same time there is pallor of the skin, mucous membranes, decreased appetite, imbalance in behavior, loss of pure first tone at the apex of the heart, “spinning top noise” on the vessels of the neck, moderate liver enlargement. In the blood, a decrease in hemoglobin level is observed to 80.3 g/l (8.03 g%). The number of erythrocytes is less than 3.6-10/l, the color index is 0.7-0.75.

In severe anemia, the child's general condition is significantly impaired. The outer integument is pale, the skin is waxy or yellowish, dry; the eyelids and feet are swollen, cracks in the corners of the mouth and on the mucous membrane of the border of the lips can be pinpoint, with sometimes more significant hemorrhages on the torso, face, and limbs. There is flabbiness of the muscles. Hyperplasia of peripheral lymph nodes and tonsils is pronounced. The abdomen is usually distended, the liver and spleen are significantly enlarged. At rest in children, tachycardia, shortness of breath, and systolic heart murmur are detected. Appetite is sharply reduced to the point of anorexia. There may be vomiting and loose stools.

On the part of red blood, a decrease in hemoglobin level is recorded below 60.6 g/l (6.06 g%), sometimes up to 20-30 g/l (2.0-3.0 g%), the number of red blood cells is less than 3-1012 - 2.5 10 12 / l (3-2 million/mm3), sometimes 1.8-1012-1.5. Yu/l, color index 0.4-0.5.

Treatment. Therapy for anemia should include comprehensive measures to eliminate etiological factors and associated conditions (rickets, malnutrition, exudative diathesis, etc.), as well as special methods of anti-anemic treatment. When implementing all these measures, a differentiated individual approach to each child is necessary, taking into account the duration of the disease and the severity of anemia.

A specific method of treating iron deficiency anemia is the administration of iron supplements.

The most commonly used iron supplements for anemia are prescribed in the following dosages (Table 16).

To avoid possible side effects (nausea, vomiting, frequent loose stools, constipation, abdominal pain), iron supplements are prescribed at half the daily dose during the first few days, and then quickly switched to a full dose according to age.

Iron supplements should be taken during or after meals along with ascorbic acid.

You can give iron supplements with citrus juices, which eliminate unpleasant taste sensations and improve the absorption of the administered medication. Iron supplements are taken for 1-2 months.

Taking into account multivitamin deficiency in these diseases, vitamins are introduced into the complex of antianemic therapy. Initially, vitamins Bi and C are prescribed daily or every other day; the course of treatment consists of 10-20 intramuscular injections. Thiamine bromide in a 5% solution is dosed at 0.3-0.5 ml, ascorbic acid in a 5% solution - 1 ml. This achieves increased gastric secretion, an increase in the child’s appetite and improved absorption of dietary iron.

Table 16 Zhelgze preparations used for the treatment of anemia in young children (single doses)

As appetite appears, in order to regulate protein metabolism and have a beneficial effect on erythropoiesis, it is advisable to use vitamin Be in the form of a 2.5% solution in a dose of 0.5-1 ml intramuscularly; course of treatment - 10-20 injections.

In cases where there is unstable stool, for better absorption of iron it is necessary to take iron supplements together with folic acid. Children 1 year of age are prescribed 0.0005 g of folic acid 3 times a day, children aged 2 to 5 years - 0.002-0.004 g 2-3 times a day.

The therapeutic effect occurs in a short time and is more pronounced when iron supplements are used together with vitamin Biz (cyanocobalamin). It is administered in a dose of 30-50 mcg intramuscularly daily or every 1-2 days, depending on the patient’s condition. The course of treatment requires 10-15 injections.

In severe forms of anemia and in the presence of severe concomitant diseases, along with iron supplements and anti-aemic vitamins, repeated blood transfusions (whole blood, red blood cells, blood plasma, etc.) are indicated. Transfusions are performed at the rate of 7-10 ml of blood per 1 kg of body weight at intervals of 5-7 days, a total of 6-8 transfusions.

Among therapeutic measures, an important place is occupied by a rationally constructed diet and the correct daily routine of a child suffering from anemia.

Children in the first six months of life with the development of anemia should receive breast milk, they are promptly introduced to fruit or vegetable juices and physiological complementary foods (vegetable, fruit puree, egg yolk, cottage cheese, porridge); When artificial feeding, preference is given to kefir and its dilutions.

In the daily diet of children in the second half of the year and the second year of life, it is necessary to increase proteins by 10-15% due to the additional introduction of cottage cheese and meat while limiting fat by 10-15%.

The diet should include foods rich in iron, copper, cobalt, nickel and manganese: oatmeal, buckwheat, green peas, potatoes, beets, radishes, cabbage, nuts, black currants, plums, pears, apples, dried fruits.

It is necessary to additionally include in the diet such products as cottage cheese, egg yolk, liver, yeast, as they are rich in cobalt, cyanocobalamin, folic acid - substances necessary for building the stroma of blood cells and globin.

When organizing the regimen of children with anemia, it is necessary to take into account that they easily become tired, therefore additional daytime sleep and sufficient duration of night sleep are necessary measures in complex treatment.

Much attention is paid to the clothing of such patients, taking into account significant disturbances in thermoregulation processes with a tendency to rapid cooling. Clothes should be warm enough, but not restrict movement or impede blood circulation. Children should definitely take walks, of course, taking into account specific weather conditions. In winter, children are allowed to stay outdoors at a temperature not lower than -15 ° C in the absence of wind. The air temperature in the room or ward should be within 20-22 °C.

In all other respects, activities aimed at enhancing physical development and increasing the body’s resistance must be carried out taking into account the age and individual characteristics of the child’s reactivity in each specific case.

Treatment of children with mild forms of anemia can be carried out at home, as well as in nurseries, kindergartens, and orphanages.

Children with severe forms of anemia are usually treated in a hospital setting, if possible hospitalized in separate small rooms to avoid the addition of certain diseases.

Prevention of nutritional-infectious anemia should be carried out in the antenatal period. It comes down to strengthening the health of a pregnant woman, combating toxicosis, and taking measures to prevent prematurity and post-maturity.

From the age of one month, the child must be given juices (apple, grape, blackcurrant, orange) in gradually increasing doses (maximum 10 teaspoons) to timely replenish the body with microelements, mineral salts, and vitamins. A very important point is the timely introduction of complete complementary foods, as well as maximum protection of children from acute infectious diseases, especially pneumonia, acute respiratory and gastrointestinal diseases that contribute to anemia in the body.

Children aged 3-6 months, who are prone to anemia, require special attention when carrying out preventive measures. Children from multiple pregnancies, premature, post-term children, with intrauterine malnutrition, children from mothers who suffered from anemia during pregnancy, from mothers with placental abnormalities should be prophylactically prescribed iron supplements together with ascorbic acid. Ascorbic iron, ferric gluconate, ferroaloe - the most commonly used drugs - are prescribed according to one-third and one-third of the daily therapeutic dose for 1-2 months.

The same contingent of children needs periodic testing of red blood (at 3, 6 months and 1 year).

It is necessary to systematically monitor the state of red blood in children's groups (nurseries, kindergartens, orphanages) and if hematological abnormalities are detected (hemoglobin content below 108 g/l, number of red blood cells less than 4.5 10 12 /l), treatment and preventive measures should be carried out .

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Blood diseases constitute a large and heterogeneous group of syndromes that develop when there are disturbances in the qualitative and quantitative composition of the blood. The practical direction that develops the principles of diagnosis and treatment of blood diseases is hematology and its separate branch - oncohematology. Specialists who correct the condition of the blood and hematopoietic organs are called hematologists. Hematology has the closest interdisciplinary ties with internal medicine, immunology, oncology, and transfusiology.

Since ancient times, in many cultures, human blood has been endowed with mystical properties, symbolizing the divine source and flow of life. “Precious”, “hot”, “innocent”, “young”, “royal” - whatever properties people endowed with blood, and the epithet “blood” always meant the highest degree of certain manifestations - consanguinity, blood enemy, blood resentment , blood feud.

Meanwhile, from the point of view of physiology, blood is a liquid medium of the body, continuously circulating through the vascular system and performing a number of important functions - respiratory, transport, regulatory, protective, etc. Blood consists of a liquid fraction - plasma and formed elements suspended in it - blood cells (erythrocytes, platelets and leukocytes). The main organs of hematopoiesis (hemocytopoiesis), which are a kind of “factory” for the production of blood cells, include red bone marrow, thymus, lymphoid tissue and spleen. Blood diseases are spoken of when there is a violation of the morphology or number of certain blood cells or changes in the properties of plasma.

All diseases of the blood and hematopoietic system are classified based on the damage to one or another of its components. In hematology, blood diseases are usually divided into three large groups: anemia, hemoblastosis and hemostasiopathy. Thus, frequent anomalies and lesions of erythrocytes include deficiency, hemolytic, hypo- and aplastic anemia. The structure of hemoblastoses includes leukemia and hematosarcoma. Blood diseases associated with damage to the hemostatic system (hemostasiopathy) include hemophilia, von Willebrand disease, thrombocytopathies, thrombocytopenia, disseminated intravascular coagulation syndrome, etc.

Blood diseases can be congenital or acquired. Congenital diseases (sickle cell anemia, thalassemia, hemophilia, etc.) are associated with gene mutations or chromosomal abnormalities. The development of acquired blood diseases can be triggered by numerous environmental factors: acute and chronic blood loss, exposure of the body to ionizing radiation or chemical agents, viral infections (rubella, measles, mumps, influenza, infectious mononucleosis, typhoid fever, viral hepatitis), nutritional deficiency, impaired absorption of nutrients and vitamins in the intestines, etc. When bacterial or fungal agents penetrate into the blood, a serious blood disease of infectious origin occurs - sepsis. Many blood diseases go hand in hand with collagenoses.

Manifestations of blood diseases are many-sided and not always specific. Characteristic signs of anemia include unreasonable fatigue and weakness, dizziness and even fainting, shortness of breath during exercise, and pale skin. Blood coagulation disorders are characterized by petechial hemorrhages and ecchymosis, various types of bleeding (gingival, nasal, uterine, gastrointestinal, pulmonary, etc.). In the clinic of leukemia, intoxication or hemorrhagic syndromes come to the fore.

The very first method with which the diagnosis of blood diseases begins is the study of a hemogram (clinical analysis) with determination of the quantitative composition of the blood and the morphology of the formed elements. For blood diseases that occur with impaired hemostasis, the number of platelets, blood clotting and bleeding time, prothrombin index, coagulogram are examined; Various types of tests are carried out - a tourniquet test, a pinch test, a jar test, etc.

To assess changes occurring in the hematopoietic organs, bone marrow puncture (sternal puncture) is used. To determine the causes of anemic syndrome, patients may need to consult a gastroenterologist and gynecologist; an FGDS, colonoscopy, and liver ultrasound.

Any changes in hemogram or myelogram, as well as symptoms indicating the likelihood of developing blood diseases, require a competent assessment by a hematologist, dynamic observation or specialized treatment under his supervision. Modern hematology has developed the fundamental principles of treatment for various blood diseases and has accumulated vast experience in curing them. If possible, treatment of blood diseases begins with eliminating the causes and risk factors, correcting the functioning of internal organs, replenishing missing substances and microelements (iron - for iron deficiency anemia, vitamin B12 - for B12-deficiency anemia, folic acid - for folate deficiency anemia, etc. .).

In some cases, taking corticosteroids, hemostatic drugs, and extracorporeal hemocorrection (erythrocytapheresis) may be indicated. Hematological patients often require transfusions of blood and its components. The most relevant and effective methods for treating hematoblastoses around the world today are cytostatic therapy, radiotherapy, allogeneic and autologous bone marrow transplantation, and the introduction of stem cells. A number of blood diseases (thrombocytopenic purpura, autoimmune anemia, myeloid leukemia, etc.) are an indication for removal of the spleen - splenectomy. Treatment of blood diseases in Moscow is carried out in specialized hematological medical and scientific centers with world-class technical equipment and highly qualified specialists.

» you will be able to find answers to basic questions regarding blood diseases, get acquainted with the main nosological forms, symptoms, principles of diagnosis and treatment.

1. ANEMIA SYNDROME (GENERAL ANEMIC).

Definition: A symptom complex caused by a decrease in hemoglobin and red blood cells per unit volume of blood with a normal or reduced volume of circulating blood.

Causes: Blood loss (acute and chronic). Impaired blood formation (deficiency or inability to use iron, vitamins (B 12 and folic acid); hereditary or acquired (chemical, radiation, immune, tumor) damage to the bone marrow. Increased blood destruction (hemolysis).

Mechanism: Reduction of hemoglobin functioning in the body - hypoxia - compensatory activation of the sympathoadrenal, respiratory and circulatory systems.

Complaints: General weakness, dizziness, shortness of breath, palpitations, tinnitus.

Inspection. Paleness of the skin and mucous membranes. Dyspnea. Palpation, weak pulse, rapid, thread-like. Decreased blood pressure.

Percussion: Expansion of relative cardiac dullness to the left (anemic myocardial dystrophy).

Auscultation. Heart sounds are muffled and rapid. Systolic murmur at the apex of the heart and large arteries. Laboratory data:

In the general blood test: a decrease in the content of erythrocytes and hemoglobin, an increase in POP. Depending on the etiology and taking into account the color indicator, anemia can be hypochromic, normochromic, hyperchromic.

2. TISSUE IRON DEFICIENCY SYNDROME.

Definition: Combines symptoms caused by a lack of jelly in tissues, excluding hematopoietic tissue.

Causes: Chronic blood loss, increased breakdown of iron (pregnancy, lactation, growth period, chronic infections, tumors), impaired iron absorption (gastric resection, enteritis), iron transport.

Mechanism: Iron deficiency is a disruption of the activity of numerous tissue iron-containing enzymes.

Complaints: Decreased appetite, difficulty swallowing, perversion of taste - addiction to chalk, lime, coal, etc.

Examination: Smoothness of the tongue papillae. Dry mucous membranes. Dry, brittle hair. Striations, brittleness and changes in the shape of the nails. Cracks in the corners of the mouth.

Palpation: Dry skin, peeling.

Percussion: Expansion of relative cardiac dullness to the left.

Auscultation: Heart sounds are muffled and rapid.

Laboratory data: In the blood: Decreased serum iron levels, increased total iron-binding capacity of serum.

General blood test: hypochromic anemia, microcytosis, anisocytosis, poikilocytosis.

Instrumental research.

Esophagogastrofibroscopy: atrophic gastritis.

Study of gastric juice: decreased gastric secretion (basal and stimulated).

3. HEMOLYSIS SYNDROME.

Definition: A symptom complex caused by increased breakdown of red blood cells.

Causes: Congenital diseases with changes in the shape of red blood cells (microspherocytosis, thalassemia, sickle cell anemia); paroxysmal nocturnal hemoglobinuria, march hemoglobinuria, poisoning with hemolytic poisons, heavy metals, organic acids; malaria; immune hemolytic anemia.

Mechanism:

a) increased breakdown of red blood cells in the spleen cells - increased formation of indirect bilirubin,

b) the breakdown of red blood cells inside the vessels - the entry of free hemoglobin and iron into the blood plasma.

Complaints: Darkening of urine (constant or paroxysmal), pain in the left hypochondrium, possible chills, vomiting, fever, intense coloration of stool.

Examination: Jaundice discoloration of the skin and mucous membranes.

Palpation: Enlargement mainly of the spleen, to a lesser extent - of the liver.

Laboratory data:

In blood plasma: the content of indirect bilirubin or free hemoglobin and iron is increased.

In the blood: an increase in reticulocytes, pathological forms of red blood cells, a decrease in the osmotic stability of red blood cells; normal color indicator.

In urine: increased content of stercobilin or hemosiderin. To exclude the immune etiology of hemolysis, the Coombs test and the hemagglutination aggregate test (detection of antibodies to red blood cells) are used.

4. HEMORRHAGIC SYNDROME.

Definition: A complex of symptoms based on increased bleeding.

Causes: Thrombocytopenic purpura (immune origin, or symptomatic thrombocytopenia with suppression of proliferation of bone marrow cells (aplastic anemia), with replacement of bone marrow with tumor tissue (hemoblastosis, tumor metastases in the bone marrow), with increased consumption of platelets (DIC syndrome), with a deficiency vitamin B 12 or folic acid); thrombopitopathy (usually a hereditary disorder of platelet function); hemophilia (hereditary deficiency of 8, or 9, or 11 plasma coagulation factors), acquired coagulopathies (deficiency of plasma coagulation factors in many infections, severe enteropathies, liver damage, malignant neoplasms); hemorrhagic vasculitis (immune inflammatory damage to blood vessels); hereditary disorder of the vascular wall of a separate localization (Rendu-Osler telangiectasia), hemangiomas (vascular tumors).

Mechanism:

I. Decrease in the number of platelets or their functional inferiority;

P. Deficiency of coagulation factors in plasma (coagulopathy);

Sh.Damage to the vascular wall of an immune or infectious-toxic nature (vasopathy).

These 3 mechanisms correspond to 3 variants of hemorrhagic

syndrome (see below):