Diseases of the hematopoietic organs. anemia. Diseases of the blood and hematopoietic organs Diseases of the group of hemoblastoses

Anemia

The term "anemia" refers to pathological conditions characterized by a decrease in the content of hemoglobin (Hb) and / or the number of erythrocytes (ER) per unit volume of blood.

Anemia syndrome is detected at any age and is one of the most common pathologies. If we take into account all anemias not only as nosological forms, but also as an anemic syndrome in various diseases, then the scale of the problem is so wide that it is sometimes characterized as a “hidden epidemic” (“Anemia is a hidden epidemic”, 2004). Anemia is detected in 15-20% of pregnant women, and according to some data - in 40% of expectant mothers.

Depending on the level of hemoglobin, severe anemia (hemoglobin level 75 g/l and below), moderate or moderate (hemoglobin 80-100 g/l) and mild (100-110 g/l) anemia are distinguished.

Anemia is also divided into groups depending on a number of signs:

• Etiologically they are divided into anemia due to intra-erythrocyte factors - usually congenital (membrane abnormalities, fermentopathy, hemoglobinopathies), and anemia due to extra-erythrocyte factors - usually acquired.
• Depending on the size of red blood cells- microcytic anemia (mean volume of erythrocytes MSU< 80 мкм 3), нормоцитарные (СДЭ = 7-8 мкм;МСУ = 80-100 мкм 3) и макроцитарные (МСУ более 95-100 мкмЗ) МСУ анемии.

• Depending on the degree of saturation with hemoglobin - hypochromic (with a color index - CP - less than 0.85 and an average concentration of hemoglobin in erythrocytes - MCHC - below 30 g / dl), normochromic (CC = 0.9-1.1; MCHC = 30-38 g / dl) and hyperchromic (CP above 1.1; MCHC more than 38 g / dl) anemia.
• Depending on the safety and adequacy of the bone marrow response on a decrease in the level of hemoglobin and erythrocytes, determined by the number of reticulocytes, anemia can be divided into hyporegenerative (with a reticulocyte level of less than 1-1.2% in the presence of anemia), associated with a violation of the production of erythrocytes in the bone marrow, as well as normo- or hyperregenerative ( the level of reticulocytes is increased moderately or significantly - up to 20-30% or more.An increase in the number of reticulocytes indicates that anemia is most likely due to hemolysis (ie, increased destruction of red blood cells) or bleeding.

Taking into account the leading mechanism of development, pathogenetic classifications are built, an example of which may be the following variant of grouping anemia according to the pathogenetic mechanism (Vorobiev P. A., 2001):

1. iron deficiency anemia.
2. Anemia associated with impaired synthesis topic: sideroachrestic, deficiency of hemsynthetase.
3. Anemia associated with a violation of DNA synthesis - megaloblastic anemia.
4. Anemia due to a violation of iron transport - atransferrinemia.
5. hemolytic anemia.
6. Anemia associated with bone marrow failure.
7. Anemia associated with dysregulation of erythropoiesis (increased levels of erythropoiesis inhibitors).

Clinical manifestations anemia

depend on the degree of decrease in the oxygen-saturating capacity of the blood, the degree of change in the total blood volume, the manifestations of the underlying disease, which leads to the development of anemia, and the ability of the cardiovascular and respiratory systems to compensate for anemia.

The diverse clinical and hematological manifestations of anemia can be divided into two main groups: symptoms, the occurrence of which is associated with hypoxia (the so-called non-specific symptoms) and symptoms that are characteristic only for a certain anemia.

The general anemic symptoms that make up the general anemic syndrome include weakness, pallor of the skin and mucous membranes, shortness of breath, tachycardia, dizziness, headache, decreased mental concentration, and drowsiness. Almost all types of anemia are characterized by symptoms from the cardiovascular system, which are manifested by the presence of a heart murmur, usually systolic in nature, which is heard in the pulmonary artery. In severe anemia, murmurs can be detected in the area of ​​the mitral and tricuspid valves. These murmurs are easily differentiated from murmurs arising from organic lesions of the heart. With anemia, a gallop rhythm of the presystolic and protodiastolic types is often observed. ECG changes are manifested in the depression of the 8T interval with a 17-shaped deformation of the 8T segment, a change in the duration of the electrical systole (C>T interval), and a violation of atrioventricular conduction. In severe anemia (Hb level below 60-70 g/l), atrial fibrillation may occur.

When diagnosing anemia, it is important to find out the features of the onset of the disease. So a gradual onset is more often associated with a violation of the production of red blood cells, an acute one is more often observed with increased destruction of red blood cells. It should be noted that there were provoking factors (viral infections, chemical and physical factors, etc.), which may indicate in favor of a certain type of anemia (autoimmune, enzyme pathologies, etc.).

To establish the pathogenesis of anemia, when assessing "red blood" indicators, attention is paid to the so-called erythrocyte parameters (indices), reflecting the size of erythrocytes and their degree of saturation with hemoglobin, the number of reticulocytes and the morphological characteristics of erythroid cells, which are noted by a laboratory assistant when viewing a blood smear.

A decrease in MSU is typical for microcytic - iron deficiency anemia (IDA), thalassemia. The cause of macrocytic anemia, characterized by an increase in MSU, may be megaloblastic anemia or disorders not associated with impaired DNA synthesis. So the cause of macrocytosis can be chronic liver disease, chronic kidney disease, smoking, hypo- and hyperthyroidism.

Hypochromia of erythrocytes is detected in cases where the decrease in Gb is more pronounced than the decrease in the number of Er. Most often this occurs with violations of the processes of hemoglobin synthesis (with iron deficiency anemia, thalassemia, lead poisoning) and sideroblastic anemia (impaired utilization of iron stores). As normochromic, hemolytic anemias and anemias associated with a hypoplastic state of the bone marrow in particular are usually characterized. Hyperchromia - increased hemoglobin saturation of the cytoplasm of cells is characteristic of macro- and megalocytes.

Hyporegenerative anemias with reduced or normal reticulocyte levels are seen with iron deficiency, anemia of chronic disease, or myelodysplasia. A significant increase in the number of reticulocytes indicates that anemia is most likely due to hemolysis or bleeding.

Important information can be obtained by assessing the morphological features of erythrocytes. The presence of macro- and especially megalocytosis of erythrocytes is typical for B p and folic deficiency anemia. Spherocytes are found in autoimmune hemolysis or hereditary spherocytosis, schizocytes are fragmented erythrocytes split by fibrin filaments in microangiopathies (thrombotic thrombocytopenic purpura or disseminated intravascular coagulation - DIC). Target-shaped (“targeted”) cells appear in a small amount in the blood in a number of hemoglobinopathies, in liver pathology, but are most characteristic of thalassemia, in which their percentage can be significant. The appearance of basophilic puncture of erythrocytes is characteristic of lead poisoning, thalassemia, and other dyserythropoietic anemias.

Nuclear forms of erythrocytes (normoblasts or erythrokaryocytes) are observed in erythroblastic anemia, bone marrow infiltration, hemolysis, and hypoxia.

Further studies are being carried out to clarify the alleged variant of anemia and include biochemical, immunological and other types of tests.

There are certain groups of patients who are at risk for the development of one or another type of anemia, which it is desirable to examine regularly as a screening in order to identify a predisposition to anemia or early stages of anemia and to carry out appropriate preventive and therapeutic measures.

Iron-deficiency anemia

Iron deficiency anemia (IDA) is the most common form of anemia. The social significance of this pathology is determined by the frequent occurrence of WDN among women of childbearing age and children, the adverse effect of iron deficiency on the growth and development of children and adolescents, a decrease in working capacity and a deterioration in the quality of life of adults, and the dependence of the incidence rate on a number of social factors (standard of living, education, healthcare).

The main reasons for the development of an imbalance of iron metabolism in the body, leading to iron deficiency states:

• Blood loss, especially menorrhagia or bleeding from the gastrointestinal tract (GIT) with esophagitis, peptic ulcer, carcinoma, colitis, diverticulitis, hemorrhoids.
• Inadequate nutrition leading to the development of IDA in children and adolescents, less often in adults.
• Worm infestations and associated GI blood loss.
• Malabsorption (for example, in intestinal diseases).

Groups at increased risk of developing iron deficiency include:

• Children: Fast growing iron needs often exceed supply.
• Girls in adolescence.
• Women: uncompensated iron loss during menstruation, pregnancy, childbirth, hyperpolymenorrhea.
• Donors without compensation for iron losses.
• The elderly due to chronic gastrointestinal diseases and a diet containing little meat products. Helicobacter pylori infection plays a certain role in the development of the disease.

Clinical picture the disease consists of nonspecific manifestations of general anemic syndrome and manifestations of tissue iron deficiency - the so-called sideropenic syndrome. As a rule, dry skin is noted, a characteristic alabaster or greenish tint of the skin, as well as a bluish tint of the sclera (“blue sclera symptom”), as a reflection of dystrophic changes in the cornea in conditions of iron deficiency, increased fragility of nails and hair. Perhaps the appearance of a transverse striation of the nail plate and their specific "spoon-shaped" changes - koilonychia. Patients have severe general weakness, which may not correspond to the degree of anemia, and muscle weakness due to impaired myoglobin synthesis. Dysphagia, perversion of taste and smell with a predilection for unusual odors, "seizures" in the corners of the mouth (angular stomatitis), smoothness of the papillae of the tongue, dysuric phenomena, urinary incontinence during laughter, coughing can be detected.

Iron deficiency anemia is accompanied by numerous complications during pregnancy and childbirth, both in the mother and the fetus, including miscarriage, bleeding during childbirth.

Since the disease develops slowly (months and even years), clinical manifestations are usually smoothed out and patients are adapted to many manifestations.

Blood tests for IDA are characterized by the presence of hypochromic microcytic anemia, anisocytosis of erythrocytes is noted. When evaluating a blood smear, the pallor of erythrocytes attracts attention, there are erythrocytes in the form of rings with a wide enlightenment in the center (anulocytes). With deep anemia, pronounced anisocytosis and poikilocytosis of erythrocytes are noted, single target cells may appear. The number of reticulocytes is usually normal, because. the regenerative capacity of the erythroid germ of the bone marrow is preserved. Transient reticulocytosis can occur with severe blood loss or when taking iron supplements shortly before the tests. In some patients, moderate leukopenia is possible and thrombocytopenia (more often in children) or thrombocytosis may be noted.

IDA is diagnosed with a low level of serum iron (<12 мкмоль/л) и снижении ферритина сыворотки (более информативный по­казатель в отношении общего содержания железа в организме) в сочетании с по­вышенной общей железосвязывающей способностью сыворотки >69 µmol/l (OZhSS). Percent saturation of transferrin with iron (Knas)<17% (Ы = 25^5). Различают следующие стадии развития заболевания:

• Prelatent Re deficiency - depletion of Re stores without clinical manifestations.
• Latent Re deficiency - delayed synthesis of the theme, the appearance of erythrocyte hypochromia, a tendency to microcytosis, hypoferremia, a reduced number of sideroblasts in the bone marrow. Clinical signs of sideropenia appear.
• Manifest ZhDA. Importance is attached to the detection of iron deficiency at the earliest stages. A decrease in the level of serum ferritin below 12 μg / l, a decrease in the excretion of iron in the urine in the desferal test less than 0.4-0.2 mg and a decrease in the number of sideroblasts (iron-containing bone marrow cells) in the sternal punctate to 15% or less are considered reliable signs of latent iron deficiency . An increase in the values ​​of the index CG)\U is detected at an early stage of WDN. The appearance of reticulocytes with a low content of Hb (CH< 26 р§) повышение уровня растворимых трансферриновых рецепторов-рТФР (кТЙК. — зо1иЫе ТгК) — ранние предикторы железодефицита. Однако методики определе­ния данных показателей недоступны для большинства лабораторий или требуют наличия специальных моделей гематологических анализаторов.

Differential Diagnosis It is carried out, first of all, with other hypochromic anemias, which include anemia with impaired hemoglobin synthesis due to causes other than true iron deficiency. Rare variants in our regions are hemoglobinopathies, in particular, thalassemia, which has a corresponding family history and is accompanied by signs of hemolysis and a characteristic morphology of erythrocytes, the presence of pathological hemoglobin fractions. Infrequent variants of hypochromic anemia are also sideroblastic anemia, anemia with lead intoxication. More often there is a need for differential diagnosis, especially in the elderly, with anemia of chronic diseases (ACD).

Therapy begins with the identification and elimination of the cause of the disease. The basis of the treatment of IDA proper is iron replacement therapy. The main goal of therapeutic measures is a stable complete cure for WDN.

Preference is given to oral iron preparations. The need for the use of parenteral (intramuscular, intravenous) iron preparations rarely occurs: with severe malabsorption, bowel resection. Blood transfusions for IDA are carried out only for health reasons.

Prevention of iron deficiency anemia

In order to primary prevention individuals with an increased predisposition to iron deficiency and the development of IDA should be the object of active attention of health care workers. Active clinical examination in risk groups can reduce the number of relapses and disability cases by 5-20 times. Recommendations for persons at risk can be represented by the following provisions:

• Eating well and varied.
• Monitor changes in the state of your health and consult a doctor in a timely manner at the first signs of trouble, including general weakness, drowsiness, and an increased tendency to "cold" diseases.
• Know your hemoglobin. All those belonging to the "risk groups" (women with prolonged "menstruation", women after childbirth, the elderly, etc.) conduct a laboratory study with the determination of the hemoglobin index at least twice a year.
• If menstruation lasts five or more days, consult a gynecologist to find out the cause and eliminate it.
• Timely treat diseases that are accompanied by bleeding from the stomach, intestines, nose, etc. It should be noted that in patients who take acetylsalicylic acid and other non-steroidal anti-inflammatory drugs for a long time, the likelihood of chronic blood loss from the gastric mucosa increases. Such blood loss, potentially leading to IDA, is latent and requires the active administration of diagnostic tests such as fecal occult blood testing (Gregersen's test) to be detected.
• Women who want to give birth to a healthy child and maintain their health should adhere to the intervals between births. To prevent iron deficiency anemia, this period should be 3-5 years.
• During pregnancy, starting from the 2nd trimester, prophylactic administration of iron preparations is prescribed.
• Prophylactic iron intake is also indicated for most active blood donors.

secondary prevention. In cases of successful treatment of IDA, when the cause of iron imbalance cannot be completely eliminated (preservation of polymenorrhea, gastrointestinal pathology with impaired iron absorption, etc.), short courses of iron-containing preparations are recommended to prevent recurrence of the disease.

Doses of iron-containing preparations used for prophylactic purposes are usually! / 2 daily therapeutic doses. Preparations based on the polymaltose complex have a certain advantage over salt preparations during preventive courses, since when they are taken, the risk of overdose is minimized.

Anemia of chronic disease

Anemia of chronic diseases (ACD) or anemia of chronic inflammation (ACH) is a type of anemia that accompanies chronic infections, inflammatory diseases and neoplastic processes, which is characterized by a decrease in the production of red blood cells in the bone marrow while maintaining iron stores in the body.

ACD is a fairly widespread pathology and ranks second after IDA among all forms of anemia. In the group of elderly people, the share of ACD reaches 30-50%. Among patients with chronic kidney diseases, especially those accompanied by renal insufficiency, ACD is registered in 25-50% of patients. ACD occurs in systemic connective tissue diseases, osteomyelitis, tuberculosis, subacute bacterial endocarditis, and tumor diseases.

Characteristic for this pathology is a violation of the use of iron compounds by the body with a reduced release of iron from macrophages and the presence of iron in the reticuloendothelial system of the bone marrow (HES) with a reduced amount in erythroid precursors. In the pathogenesis of the disease, dysregulation of cellular metabolism and erythropoiesis under the influence of pro-inflammatory cytokines, a decrease in the sensitivity of bone marrow erythroid precursors to erythropoietin and a deficiency of erythropoietin itself, increased consumption of iron by non-eryphoid cells, and other disorders play a role.

In the clinical picture the symptoms of the underlying disease predominate in combination with manifestations of anemic syndrome of varying severity.

Anemia is usually normochromic in nature, the average diameter and average volume of erythrocytes are normal. As the disease progresses, anemia acquires the character of hypochromic microcytic, which requires differential diagnosis with IDA. Elevated levels of iron and ferritin in serum testify in favor of ACD. The level of reticulocytes is normal or slightly reduced. Often there is an increase in ESR. Often there are dysproteinemia, an increase in plasma C-reactive protein, haptoglobin, ceruloplasmin, the C3 component of complement, as a reflection of a chronic inflammatory or tumor process. The content of serum erythropoietin can be moderately increased, the level of total iron-binding capacity (TIBC) decreases.

In difficult diagnostic cases, a bone marrow examination is performed. Characteristic of ACD is the presence of adequate or increased iron stores in HES cells, that is, the number of sideroblasts in the bone marrow is normal or increased.

The effectiveness of ACD therapy largely depends on the success of the treatment of the underlying disease. In a number of diseases (rheumatoid arthritis, etc.), a positive therapeutic effect is obtained by the use of glucocorticoids (GC) by reducing the formation of inflammatory cytokines. In a significant part of patients, they are successfully used in relatively large doses: 100-150 IU / kg. This category of patients primarily includes patients with anemia in renal failure with a low level of endogenous Epo.

In cases of severe anemia and in the presence of severe? hypoxic syndrome, transfusions of erythrocyte mass are indicated.

Megaloblastic anemias

Megaloblastosis refers to pathological processes caused by disturbances in DNA synthesis and characterized by a delay in the maturation of the nuclei of hematopoietic progenitor cells with the continued development of the cytoplasm. The result of such nuclear-cytoplasmic dissociation is the production of cells larger than normal. The mean volume of erythrocytes is increased (MSU> 100 fl).

B | 2 -DEFICIENCY ANEMIA (B ] 2 - YES)

The disease is caused by malabsorption of B / 2 as a result of atrophic gastritis and lack of secretion of the internal gastric factor (pernicious anemia, Addison-Birmer disease), gastrectomy; alimentary deficiency (in particular, in vegetarians); sometimes diseases of the terminal ileum (Crohn's disease) or its resection; blind loop; diverticulum; helminthic infestations (Burmnobho1gshgp).

B 12 is found in the liver and all animal products. There are reserves of the vitamin in the body.

Often B 12 -DA is associated with thyroid diseases (up to 25%), vitiligo, Addison's disease, gastric carcinoma.

In the clinical picture diseases, along with general anemic symptoms, there may be signs of damage to the central and peripheral nervous system, gastrointestinal tract, which is manifested by such disorders as: paresthesia; peripheral neuropathy, impaired positional and vibrational sensitivity; neuropsychiatric abnormalities; glossitis - painful red tongue; diarrhea.

It is important to note that neurological symptoms (symptoms of the so-called "funicular myelosis") can outpace the development of anemia.

Possible moderate jaundice (lemon skin tone), moderate splenomegaly and bilirubinemia due to indirect fraction due to hemolysis (mainly intramedullary), often accompanying B 12 -DA. Diagnostics. The main significance in the diagnosis of V P-DA belongs to morphological studies of blood and bone marrow. Anemia has the character of macrocytic normo- or hyperchromic, hyporegenerative anemia. Aniso-, poikilocytosis, basophilic granularity of erythrocytes due to the presence of RNA elements is noted. In erythrocytes, the remains of the nucleus are found in the form of Jolly bodies, Cabot rings. In a clinical blood test, there may be leukocytopenia and thrombocytopenia, usually moderate, as well as morphological changes in granulocytes and platelets (large forms, hypersegmentation of neutrophil nuclei). To clarify the diagnosis, additional studies are indicated, including bone marrow examination to confirm the megaloblastoid type of hematopoiesis.

There are methods for determining the concentration of B ] 2 in the blood serum, which serves as a reflection of the reserves of cobalamin in the body. An indication of a clinically significant deficiency of vitamin B p is its significantly reduced serum level.

In some patients, antibodies to the parietal cells of the stomach or antibodies to the intrinsic factor (specific for pernicious anemia) can be detected. In such cases, the Schilling test is sometimes informative, which is prescribed to determine whether the deficiency of B 12 is due to malabsorption or the absence of an internal factor by comparing the proportion of the content in the oral dose (1 μg) of radioactive B | 2 with excreted in the urine - with and without additional administration of internal factor a. The concentration of homocysteine ​​in patients with B12 deficiency and folate deficiency is increased.

Differential Diagnosis carried out with other types of anemia, primarily macrocytic, as well as with folic acid deficiency anemia. The concept of macrocytic anemia reflects the increased size of red blood cells, which may be caused by disorders not related to DNA synthesis. Vitamin B12 deficiency should be distinguished from diseases such as aplastic anemia, refractory anemia, or myelodysplastic syndrome (MDS). Macrocytic anemia with pancytopenia can be caused by both hypo- and hyperthyroidism, as well as alcoholism, chronic liver diseases. Macrocytosis can be caused by chronic kidney disease and smoking. A large number of reticulocytes can increase the MSU, since reticulocytes are large cells. As a result, hemolytic anemia is sometimes mistaken for megaloblastic anemia. In difficult cases, the main method of research is the study of the bone marrow.

In treatment In p-DA, the important point is to eliminate the cause of the deficiency. Replacement therapy with cyanocobalamin is carried out until hematological parameters normalize or with CNS symptoms - until recovery is completed. Most patients require maintenance therapy with moderate doses of B 2 -DA, with the exception of neurological disorders. Maternal folate deficiency is also associated with neural tube defects in the fetus.

Diagnostics. The picture of blood and bone marrow does not differ from that in B 12 -YES.

For diagnosis and differential diagnosis, the determination of the level of folates and B 12 in serum, as well as erythrocyte folates, is used.

In mixed B 2 -folate-deficient forms of anemia or FDA misdiagnosis, the administration of folate alone may contribute to the manifestation or worsening of the course of subacute combined degeneration of the spinal cord.

Treatment. The FDA provides replacement therapy with folic acid in the form of an oral preparation. The therapy is carried out under the control of hemogram parameters (hemoglobin and erythrocyte levels, erythrocyte parameters, the appearance of a reticulocyte crisis) until the red blood parameters normalize. If it is impossible to completely eliminate the factors contributing to the development of folate deficiency, further preventive courses of therapy are carried out.

The prognosis is favorable with adequate treatment of anemia and elimination of the cause of the disease.

Prevention of folate deficiency anemia

Primary preventive interventions include monitoring individuals at risk, dietary adjustments, and prophylactic folic acid administration for diseases and conditions that favor FDA. In particular, patients with epilepsy are at risk, since anticonvulsants are potential inducers of liver enzymes, and an increase in their activity leads to an accelerated breakdown of folates and the occurrence of folate deficiency megaloblastic anemia. Therefore, patients with epilepsy and patients taking drugs from the group of antimetabolites, such as methotrexate, should be regularly examined for the timely detection of anemia and appropriate measures.

Hemolytic anemia

Hemolysis is the premature destruction of red blood cells. It can occur directly in the circulation (intravascular hemolysis) or in the reticuloendothelial system (extravascular).

Causes of hemolysis can be either genetically determined or acquired. Genetic:

1. Membrane pathology: congenital spherocytosis, elliptocytosis.
2. Pathology of hemoglobin: sickle cell disease - sickle cell anemia (SLE = SKA), thalassemia.
3. Enzyme defects: deficiency of glucose-phosphate dehydrogenase (G6PD), deficiency of pyruvate kinase, etc.

Purchased:

1. Immune: either isoimmune (hemolytic disease of the newborn, post-transfusion reactions of hemolytic type reactions), autoimmune (due to warm or cold antibodies), drug-induced.

1. Non-immune: traumatic (cardiac hemolysis, microangiopathic anemia), infectious (malaria, septicemia), membrane pathology (paroxysmal nocturnal hemoglobinuria), liver diseases.

Signs of hemolysis:

1. Clinical: yellowness of the skin, darkening of urine, hepatosplenomegaly, etc.
2. Laboratory:

- Associated with increased destruction of red blood cells:

- Increasing the level of bilirubin (unconjugated);

- Increase in the content of urobilin in the urine;

- Decrease in serum haptoglobin (binds free hemoglobin).

- Associated with increased production of red blood cells:

- Reticulocytosis;

- Polychromasia of erythrocytes;

- Hyperplasia of the bone marrow with the expansion of the erythroid germ.

When establishing the diagnosis and conducting differential diagnosis in patients with hemolytic anemia, it is necessary to pay attention to the history data (family history, nationality, jaundice, hematuria, taking drugs, previously detected anemia), icterus, hepatosplenomegaly, bone deformities (stigmas in hereditary pathology, features skulls with thalassemia, etc.), ulcers on the legs (observed with SLE, sometimes with spherocytosis).

From laboratory research indicative are a complete blood count with reticulocytes, the level of bilirubin and its fractional composition, LDH, haptoglobin (a decrease in the level is an indicator of intravascular hemolysis), urine urobilinogen. Blood smears may show polychromasia, macrocytosis, spherocytosis, elliptocytosis, fragmented or sickle cells, and target cells (characteristic of thalassemia). At the next stage, special studies are carried out, such as the Coombs test, the determination of urinary hemosiderin (indicator of chronic intravascular hemolysis). Membrane anomalies can be confirmed by osmotic stability tests. Hemoglobin electrophoresis determines hemoglobin variants. When other causes are ruled out, enzyme studies are performed.

Autoimmune hemolytic anemia (AIHA)

Autoimmune hemolytic anemia (AIHA) is an anemia in which shortened lifespan of red blood cells is the result of exposure to autoantibodies against antigens (membrane proteins) of red blood cells.

The frequency of occurrence is about 1:100,000 of the population.

Hemolysis may be due to warm or cold antibodies.

AIHA can be an independent disease or be detected in systemic connective tissue diseases, thyroid pathology, Fisher-Evans syndrome (immune dysregulation with immune thrombocyto-leukopenia, anemia in combination with a number of other disorders). Known HIV-associated AIHA, secondary AIHA due to mycoplasmal, pneumococcal infections. Perhaps the appearance of autoantibodies as a result of repeated blood transfusions, pregnancies. Cold agglutinins can be produced by mycoplasma and EBV.

Allocate acute and chronic forms. Most cases are characterized by an acute onset with a possible transition to a chronic form. Depending on the serological variant, AIHA is distinguished with complete and incomplete antibodies, with warm and cold antibodies, and hemolysin forms.

Paroxysmal cold hemoglobinuria (Donath-Landsteiner syndrome), as a rule, is observed after viral infections and in the late stages of syphilis.

In the clinical picture

symptoms of anemia and hemolysis are combined: darkening of urine, icterus of the skin and sclera, fever, abdominal pain, moderate gastosplenomegaly. A feature of cold AIHA is the exacerbation of chronic anemia in the cold, often combined with Raynaud's syndrome or acrocyanosis. Hemolysin forms are often accompanied by hemoglobinuria and other signs of acute intravascular hemolysis.

Diagnostics.

Anemia, as a rule, normochromic normocytic, characterized by reticulocytosis, often severe. Spherocytes may be present in small numbers. Possible leukocytosis with a shift of the leukocyte formula plevo, moderate thrombocytosis. Characterized by an increase in indirect bilirubin, erythroid hyperplasia of the bone marrow. Increased serum lactate dehydrogenace (LDH) levels. Serum iron levels are normal or elevated, haptoglobin levels are normal or reduced.

Differential Diagnosis

carried out with other types of anemia, primarily hemolytic, secondary anemia, Gilbert's disease. The task of general practitioners is to suspect this type of anemia and conduct a primary diagnosis. Clarification of the option and treatment is usually carried out in specialized institutions.

The main diagnostic test is a positive direct anti-globulin test (Coombs test), which detects antibodies or complement on the surface of red blood cells. Additionally, an indirect Coombs test is performed, which determines antibodies in the serum.

In treatment autoimmune forms of hemolytic anemia, the main place belongs to glucocorticosteroids (GC). In patients with acute hemolysis, intravenous immunoglobulin may be used, usually in combination with GC.

In the absence of the effect of conservative therapy, splenectomy is possible, the effectiveness of which in this pathology is about 70%. Of the immunosuppressive drugs, with the ineffectiveness of conventional therapy in the treatment of AIHA, azathiaprine, cytostatics (vinca alkaloids, cyclophosphamide), cyclosporine A are used.

The basis of the treatment of symptomatic anemia is the treatment of the underlying disease.

Prevention of autoimmune hemolytic anemia

Primary preventive interventions consist in the treatment of underlying diseases in which AIHA can occur.

secondary prevention. In order to prevent an increase in hemolysis and the development of hemolytic crises, patients suffering from AIHA are advised to avoid provoking factors: hypothermia in cold forms, viral infections in all variants of the disease, etc. Patients who underwent splenectomy, given the development of immunodeficiency, are shown pneumococcal vaccine. This recommendation primarily applies to children and persons who have additional indications for vaccination (according to the epidemiological situation, etc.).

Hereditary spherocytosis (congenital spherocytic anemia, Minkowski-Schoffard disease)

Hereditary spherocytosis (NS) is a cytoskeletal anomaly caused by a violation of the spectrin structure. The result of such anomalies is the loss of the ability of erythrocytes to deform, the work of the SH + / K + - the membrane pump is disrupted, premature (not with aging) spherulation of erythrocytes, a shortening of the lifespan of red blood cells and their destruction by spleen cells. The life span of erythrocytes is shortened to 12-14 days.

It is caused by mutations in the genes encoding membrane proteins of the erythrocyte cytoskeleton. Inheritance is autosomal dominant (manifested by mild to moderate anemia) or recessive (clinically manifested in severe form).

It is characterized by hemolytic anemia, splenomegaly and the presence of spherical erythrocytes in the peripheral blood. The disease may be hidden.

Diagnosis

NS is based on the presence of characteristic morphological changes in erythrocytes and signs of hemolysis in a patient. Hemoglobin saturation of erythrocytes and serum iron levels are usually normal, except in those cases when, against the background of a long-term hemolysis, an iron deficiency condition develops in the body.

In the bone marrow there is a compensatory increase in erythropoiesis.

Differential Diagnosis

carried out with jaundice of another etiology (infectious hepatitis, obstructive jaundice, Gilbert's syndrome, etc.), immune hemolytic anemia, microangiopathic hemolytic anemia, splenomegaly of another etiology. In differential diagnosis, along with the identification of morphologically altered erythrocytes, a negative Coombs test and other laboratory data, a carefully collected family history and examination of the patient's relatives to identify signs of NS can be of no small importance.

Treatment.

With a clinically compensated state of the patient, the absence of significant hemolysis and anemia, therapy is usually limited to symptomatic agents, including those aimed at preventing the development of cholelithiasis (cholagogue, herbal medicine, rational diet). In severe hemolysis with severe anemia and in aplastic crises with low hemoglobin levels, red blood cell transfusions are performed.

One method of therapy in patients with spherocytic anemia is splenectomy. Surgical treatment is indicated for patients with moderate to severe hemolytic anemia or its complications, including those with cholelithiasis, especially in young people. As a result of the removal of the spleen, hemolysis of erythrocytes stops or significantly decreases, and their life expectancy increases.

Prevention of hereditary spherocytosis

Primary prevention

in NS, as in other hereditary diseases, is genetic counseling and family planning.

secondary prevention.

Since in a significant part of patients the disease occurs in a latent or clinically compensated form, the main measures for secondary prevention are aimed at eliminating the manifestations of chronic intoxication, compensating for the increased consumption of substances necessary for hematopoiesis and preventing complications such as the early development of gallstone disease. In this regard, good nutrition, taking multivitamins with microelements, choleretic agents, and annual ultrasound monitoring of the state of the biliary tract are indicated.

As with other forms of chronic hemolytic anemia, patients with NS often develop folate deficiency, and therefore folic acid is prescribed prophylactically for this category of patients.

Hypoplasia of hematopoiesis

Anemia can be caused by a suppressed (hypostatic) state of hematopoiesis due to toxic and radiation effects, the development of reactive fibrosis in the bone marrow in a number of diseases, or as a result of independent diseases - hypoplastic (aplastic) anemia, partial red cell aplasia.

aplastic anemia

Aplastic anemia is a severe disease of the hematopoietic system, which is characterized by pancytopenia in the peripheral blood and hypocellular bone marrow.

The disease is rare: from 2-3 to 10-20 cases per million population per year. It is observed in all age groups. A high incidence of the disease is noted in the Far East, in Japan, and Thailand.

The causes of the development of the disease can be cytotoxic drugs, radiation, drugs (gold, chloramphenicol), industrial toxins, viruses (hepatitis). The etiological factor in half of the cases is not detected - idiopathic forms. There is a congenital form - Fanconi anemia - a genetically determined disease with hypersensitivity to DNA-damaging effects and an increased tendency to develop tumor diseases.

The modern concept of the pathogenesis of AA suggests a relationship between the development of hematopoietic aplasia and a defect in stem cells with a violation of their proliferative activity with the participation of immune-mediated mechanisms, a violation of the regulation of hematopoiesis by immunocompetent lymphoid cells.

There are acute and chronic forms of the disease, as well as severe aplastic anemia (sAA) and AA of moderate severity (non-severe aplastic anemia - nAA). TAA is determined in the presence of any 2 of the following criteria according to peripheral blood data:

1. Granulocytes less than 0.5 x 109/l
2. Platelets less than 20 x 109/l
3. Reticulocytes less than 1% (corrected for hematocrit) in combination with bone marrow aplasia according to trepanobioptates (bone marrow cellularity not more than 30% of normal).

Clinical manifestations

diseases are caused by anemic and hemorrhagic syndrome.

Diagnosis is based on the detection of characteristic changes in blood tests and bone marrow with no signs of clonal hematopoiesis. The basis of diagnosis is histological examination of the bone marrow.

Anemia of a normochromic nature, the number of reticulocytes is reduced, as a manifestation of the first shuregenerator nature of anemia.

In the mpelogram, the number of nucleated elements is reduced, the total percentage of cellular elements of granulopoiesis and erythropoiesis is reduced, a high relative number of lymphocytes is often noted, and the content of megakaryocytes is significantly reduced. In histological preparations of trephine preparations of the ilium, aplasia of the bone marrow is detected with the replacement of the hematopoietic tissue with adipose tissue.

Differential Diagnosis

it is carried out with hypoplastic variants of hemoblastoses (myelodysplastic syndrome - MDS, acute leukemia, sub-blekemic myelosis), secondary - symptomatic aplasias observed in liver diseases, a number of tumor diseases.

Treatment.

Patients with AA undergo immunosuppressive therapy, including glucocorticoid hormones (GC), antilymphocyte (ALG) or antithymocyte (ATG) immunoglobulin, cyclosporine-A (cA). The treatment of choice for tAA in patients under 40 years of age is bone marrow transplantation (BMT). Such therapy allows you to get remissions in 70-80%. Symptomatic therapy is also carried out, aimed at correcting anemic and hemorrhagic syndromes, preventing and treating possible infectious and other complications.

The prognosis of the disease primarily depends on the depth of aplasia and the severity of the disease, as well as the timeliness and activity of the therapy.

The main causes of death in patients are hemorrhagic and infectious complications, progression of aplasia with unsuccessful therapy.

Prevention of aplastic anemia

Primary preventive measures include stopping contact with factors that have hemosuppressive properties, limiting the use of drugs with myelosuppressive properties. Thus, in a number of countries, the use of the drug levomecithin (chloramphenicol) has been discontinued, since the relationship of taking this drug with an increase in the incidence of hematopoietic aplasia has been shown. With the development of AA during pregnancy, it is advisable to interrupt it.

secondary prevention.

Patients with remission of the disease should remain under observation with regular monitoring of hemogram parameters, since relapses of the disease are possible, both under the influence of adverse factors and spontaneous.

Diseases of the blood system are divided into anemia, leukemia and diseases associated with damage to the hemostasis system (blood clotting).

Causes that cause damage to the blood system.

anemia.

Among the most common causes of anemia, the following are important:

• acute blood loss (trauma);
• chronic blood loss of various localization (gastrointestinal, uterine, nasal, renal) due to various diseases;
• malabsorption in the intestine of iron, which comes with food (enteritis, intestinal resection);
• increased need for iron (pregnancy, feeding, rapid growth);
• common dietary iron deficiency (malnutrition, anorexia, vegetarianism);
• vitamin B12 deficiency (insufficient intake of it with food is meat and dairy products, malabsorption of this vitamin: with atrophic gastritis, after gastrectomy, due to hereditary factors, with the toxic effects of alcohol, with diseases of the pancreas, with wide tapeworm invasion) ;
• malabsorption of folic acid; bone marrow diseases; various hereditary causes.

Leukemias.

The reasons are not fully understood, but the following is known that it can be a hereditary predisposition, ionizing radiation, chemicals (varnishes, paints, pesticides, benzene), viruses. The defeat of the hemostasis system is most often due to hereditary factors.

Symptoms of blood diseases.

Often, patients with blood diseases complain of weakness, easy fatigue, dizziness, shortness of breath during physical exertion, interruptions in the work of the heart, loss of appetite, decreased performance. These complaints are usually manifestations of various anemias. With acute and profuse bleeding, sudden weakness, dizziness, fainting appear.

Many diseases of the blood system are accompanied by fever. A low temperature is observed with anemia, moderate and high temperature occurs with acute and chronic leukemia.

Also, patients often complain of itching of the skin.

In many diseases of the blood system, patients complain of loss of appetite and weight loss, usually especially pronounced, turning into cachexia.

For B12-deficiency anemia, patients feel a burning sensation at the tip of the tongue and its edges, with iron deficiency anemia, a perversion of taste is characteristic (patients willingly eat chalk, clay, earth, coal), as well as smell (patients experience pleasure from inhaling ether vapors, gasoline and other odorous substances with bad smell).

Also, patients may complain of various skin rashes, bleeding from the nose, gums, gastrointestinal tract, lungs (with hemorrhagic diathesis).

There may also be pain in the bones when pressed or tapped (leukemia). Often, with blood diseases, the spleen is involved in the pathological process, then there are severe pains in the left hypochondrium, and when the liver is involved, in the right hypochondrium.

There may be enlarged and painful lymph nodes, tonsils.

All of the above symptoms are a reason to see a doctor for an examination.

During the examination, the patient's condition is determined. Extremely severe can be observed in the last stages of many blood diseases: progressive anemia, leukemia. Also, on examination, pallor of the skin and visible mucous membranes is revealed, with iron deficiency anemia the skin has “alabaster pallor”, with B12 deficiency it is slightly yellowish, with hemolytic anemia it is icteric, with chronic leukemia the skin has an earthy gray tint, with erythremia - cherry red. With hemorrhagic diathesis, hemorrhages are visible on the skin and mucous membranes. The state of the trophism of the skin is also changing. With iron deficiency anemia, the skin becomes dry, flaky, hair becomes brittle, split ends.

When examining the oral cavity, atrophy of the papillae of the tongue is revealed, the surface of the tongue becomes smooth (B12 deficiency anemia), rapidly progressive tooth decay and inflammation of the mucous membrane around the teeth (iron deficiency anemia), ulcerative necrotic tonsillitis and stomatitis (acute leukemia).

Palpation reveals soreness of flat bones (leukemia), enlarged and painful lymph nodes (leukemia), an enlarged spleen (hemolytic anemia, acute and chronic leukemia). With percussion, you can also detect an enlarged spleen, and with auscultation, the noise of friction of the peritoneum over the spleen.

Laboratory and instrumental research methods.

Morphological examination of blood: general blood analysis(determination of the number of erythrocytes and the content of hemoglobin in them, determination of the total number of leukocytes and the ratio of individual forms among them, determination of the number of platelets, erythrocyte sedimentation rate). With iron deficiency anemia, the level of hemoglobin and the number of erythrocytes unevenly decrease, hemoglobin decreases more strongly. With B12-deficiency anemia, on the contrary, the number of red blood cells is reduced more than hemoglobin, and with this form of anemia, increased red blood cells can be detected. A change in leukocytes (qualitative and quantitative composition) is observed in leukemia.

Morphological evaluation of erythrocytes reveals anemia.

Puncture of hematopoietic organs. The morphological composition of the blood does not always adequately reflect the changes occurring in the hematopoietic organs. So, in some forms of leukemia, the cellular composition of the blood is almost not disturbed, despite significant changes in the bone marrow. For this, a sternal puncture is used (they take bone marrow from the sternum). Bone marrow puncture allows you to identify violations of cell maturation - an increase in the number of young forms or the predominance of primary undifferentiated elements, violations of the ratio between the cells of the red (erythrocyte) and white (leukocyte) series, changes in the total number of blood cells, the appearance of pathological forms and much more. In addition to the sternum, bone marrow can also be extracted from other bones, such as the ilium.

More accurate information about the composition of the bone marrow gives trephine biopsy when the ilium column is cut out together with the bone marrow tissue, and from which histological preparations are made. They preserve the structure of the bone marrow, and the absence of blood impurities allows you to more accurately assess it.

Enlarged lymph nodes are often punctured, while it is possible to assess the nature of changes in the cellular composition of the lymph nodes and clarify the diagnosis of diseases of the lymphatic apparatus: lymphocytic leukemia, lymphogranulomatosis, lymphosarcomatosis, detect tumor metastases and others. More accurate information can be obtained with a biopsy of the lymph node, puncture of the spleen.

A comprehensive study of the cellular composition of the bone marrow, spleen, and lymph nodes makes it possible to clarify the nature of the relationship between these sections of the hematopoietic system, to identify the presence of extramedullary hematopoiesis in some lesions of the bone marrow.

Assessment of hemolysis necessary in identifying the hemolytic nature of anemia (free bilirubin is determined, a change in the osmotic stability of erythrocytes, the appearance of reticulocytosis).

Study of hemorrhagic syndrome. There are classical coagulation tests (determination of blood clotting time, platelet count, bleeding duration, blood clot retraction, capillary permeability) and differential tests. The clotting time characterizes the clotting of blood as a whole and does not reflect the individual phases of coagulation. The duration of bleeding is determined by the Duke prick test, normally 2 to 4 minutes. Capillary permeability is determined using the following tests: tourniquet symptom (the norm is more than 3 minutes), jar test, pinch symptom, hammer syndrome and others. Differential tests: determination of plasma recalcification time, prothrombin consumption test, determination of the prothrombin index, plasma tolerance to heparin, and others. The summarized results of the listed samples constitute a coagulogram characterizing the state of the blood coagulation system. X-ray examination, it is possible to determine an increase in the lymph nodes of the mediastinum (lympholeukemia, lymphogranulomatosis, lymphosarcoma), as well as bone changes that can be in some forms of leukemia and malignant lymphomas (focal destruction of bone tissue in multiple myeloma, bone destruction in lymphosarcoma, bone compaction in osteomyelosclerosis ).

Radioisotope research methods allow to evaluate the function of the spleen, determine its size and identify focal lesions.

Prevention of blood diseases

Prevention of diseases of the blood system is as follows, it is the timely diagnosis and treatment of diseases that are accompanied by blood loss (hemorrhoids, peptic ulcer, erosive gastritis, ulcerative colitis, uterine fibromatosis, hiatal hernia, intestinal tumors), helminthic invasions, viral infection, if it is impossible to recover from them, it is recommended to take iron supplements, vitamins (especially B12 and folic acid) and, accordingly, use food containing them, and these measures should also be applied to blood donors, pregnant and lactating women, patients with heavy menstruation.

Patients with aplastic anemia need to take measures to prevent the effects of external factors on the body, such as ionizing radiation, dyes, and others. They also need dispensary observation and control over blood tests.

For the prevention of diseases of the blood coagulation system, family planning is used (prevention of hemophilia), prevention of hypothermia and stressful situations, vaccinations, tests with a bacterial antigen, alcohol (for hemorrhagic vasculitis), refusal to conduct unreasonable blood transfusions, especially from different donors, are contraindicated.

For the prevention of leukemia, it is necessary to reduce, if any, exposure to harmful factors, such as ionizing and non-ionizing radiation, varnishes, paints, benzene. To prevent serious conditions and complications, you do not need to self-medicate, but consult a doctor if any symptoms appear. If possible, try to undergo a medical examination annually, be sure to take a general blood test.

Diseases of the blood, hematopoietic organs and certain disorders involving the immune mechanism according to ICD-10

Diet related anemia
Anemia due to enzyme disorders
Aplastic and other anemias
Blood clotting disorders, purpura and other hemorrhagic conditions
Other diseases of the blood and blood-forming organs
Selected disorders involving the immune mechanism

The blood system includes:

• organs and tissues of hematopoiesis, or hematopoiesis, in which blood cells mature;
• peripheral blood, which includes fractions circulating and deposited in organs and tissues;
• organs of hemorrhage;

The blood system is the internal environment of the body and one of its integrating systems. Blood performs numerous functions - respiration, metabolism, excretion, thermoregulation, maintaining water and electrolyte balance. It performs protective and regulatory functions due to the presence in it of phagocytes, various antibodies, biologically active substances, hormones. Many factors influence the processes of hematopoiesis. Special substances that regulate the proliferation and maturation of blood cells are important, - hematopoietins, but the nervous system exerts a general regulating influence. All the numerous functions of the blood are aimed at maintaining homeostasis.

The picture of peripheral blood and bone marrow allows us to judge the functions of many body systems. At the same time, the most complete picture of the state of the hematopoietic system itself can be obtained only by examining the bone marrow. To do this, a special needle (trephine) is used to puncture the sternum or iliac crest and obtain bone marrow tissue, which is then examined under a microscope.

MORPHOLOGY OF HEMATOPOISIS

All formed elements of blood under normal conditions are formed in the red bone marrow of flat bones - the sternum, ribs, pelvic bones, vertebrae. In the tubular bones of an adult, the bone marrow is represented mainly by adipose tissue and has a yellow color. In children, hematopoiesis occurs in the tubular bones, so the bone marrow is red.

Morphogenesis of hematopoiesis.

The ancestor of all blood cells is the hematopoietic stem cell of the bone marrow, which is transformed into progenitor cells, morphologically indistinguishable from each other, but giving rise to myelo- and lymphopoiesis (Fig. 42). These processes are regulated by hematopoietins, among which erythropoietin, leuko- and thrombopoietin are distinguished. Depending on the predominance of certain poetins, myelopoiesis intensifies and progenitor cells begin to transform into blast forms of myelocytic, erythrocyte and platelet blood sprouts. With the stimulation of lymphopoiesis, the maturation of lymphocytic and monocytic blood sprouts begins. Thus, the development of mature cellular forms - T- and B-lymphocytes, monocytes, basophils, eosinophils, neutrophils, erythrocytes and platelets.

At different stages of hematopoiesis, as a result of pathological influences, violations of the maturation of hematopoietic cells may occur and blood diseases develop. In addition, the blood system reacts to many pathological processes that occur in the body by changing its cellular composition and other parameters.

BLOOD VOLUME DISORDERS

Rice. 42. Scheme of hematopoiesis (according to I. L. Chertkov and A. I. Vorobyov).

With various diseases and pathological processes, the total volume of blood, as well as the ratio of its formed elements and plasma, can change. Allocate 2 main groups of blood volume disorders:

• hypervolemia - conditions characterized by an increase in total blood volume and. usually, a change in hematocrit;
• hypovolemia - conditions characterized by a decrease in total blood volume and combined with a decrease or increase in hematocrit.

HYPERVOLEMIA

Kinds:

• Normocythemic hypervolemia - a condition manifested by an equivalent increase in the volume of formed elements and the liquid part of the circulating blood. The hematocrit remains within the normal range. Such a state occurs, for example. when transfusing a large amount (at least 2 liters) of blood.
• Oligocythemic hypervolemia - a condition characterized by an increase in total blood volume due to an increase mainly in plasma volume. The hematocrit is below normal. Such hypervolemia appears with the introduction of a large amount of saline or blood substitutes, as well as with insufficient excretory function of the kidneys.
• Polycythemic hypervolemia - a condition manifested by an increase in the total volume of blood due to a predominant increase in the number of its formed elements, primarily erythrocytes. In this case, the hematocrit becomes higher than normal. Most often, this phenomenon is observed during prolonged hypoxia, which stimulates the release of erythrocytes from the bone marrow into the blood, for example, in residents of high mountains, at certain stages of the pathogenesis of a number of lung and heart diseases.

HYPOVOLEMIA

Kinds:

• Normocythemic hypovolemia - a condition manifested by a decrease in the total blood volume while maintaining the hematocrit within the normal range, which is observed immediately after blood loss.
• Oligocythemic hypovolemia characterized by a decrease in the total volume of blood with a predominant decrease in the number of its formed elements. The hematocrit is below normal. It is also observed after blood loss, but at a later date, when tissue fluid enters the vessels from the intercellular space. In this case, the volume of circulating blood begins to increase, and the number of red blood cells remains at a low level.
• Polycythemic hypovolemia - a condition in which a decrease in total blood volume is due mainly to a decrease in plasma volume. The hematocrit is above normal. Such thickening of the blood is observed with loss of fluid after extensive burns, with hyperthermia with massive sweating, cholera, characterized by indomitable vomiting and diarrhea. Blood clotting also contributes to the formation of blood clots, and a decrease in total blood volume often leads to heart failure.

PATHOLOGY OF THE ERYTHROCYTE SYSTEM

Anemia, or anemia, - decrease in the total amount of hemoglobin in the body and, as a rule, hematocrit. In most cases, anemia is accompanied by erythropenia - a decrease in the number of erythrocytes per unit of blood volume below the norm (less than 310 9 /l in women and 410 9 /l in men). The exceptions are iron deficiency anemia and thalassemia, in which the number of red blood cells may be normal or even increased.

The significance of anemia for the body is determined primarily by a decrease in the oxygen capacity of the blood and the development of hypoxia, which is associated with the main symptoms of life disorders in these patients.

Types of anemia:

• due to blood loss - posthemorrhagic;
• due to impaired blood formation - deficient;
• due to increased blood destruction - hemolytic.

In the course of anemia can be acute and chronic.

According to changes in the structure of erythrocytes in anemia, they distinguish:

• anisocytosis, which is characterized by a different shape of red blood cells;
• poikilocytosis - characterized by different sizes of red blood cells.

Changes in anemia color indicator - the content of hemoglobin in erythrocytes, which is normally equal to I. With anemia, it can be:

• more than 1 (hyperchromic anemia);
• less than 1 (hypochromic anemia).

ANEMIA DUE TO BLOOD LOSS (POSTHEMORRHAGIC)

These anemias are always secondary, as they occur as a result of illness or injury.

Acute posthemorrhagic anemia occurs with acute blood loss. for example, from the vessels of the bottom of a gastric ulcer, with a rupture of the fallopian tube in case of tubal pregnancy, from pulmonary caverns with tuberculosis, etc. (internal bleeding) or from damaged vessels in case of injuries to the limbs, neck and other parts of the body (external bleeding).

Mechanisms of development of acute posthemorrhagic conditions. At the initial stage of blood loss, the volume of circulating blood decreases to a greater or lesser extent and hypovolemia develops. In this regard, the flow of venous blood to the heart decreases. its shock and minute ejection. This causes a drop in blood pressure and a weakening of cardiac activity. As a result, the transport of oxygen and metabolic substrates from the blood to the cells decreases, and from the latter - carbon dioxide and waste products of metabolism. Hypoxia develops, which largely determines the outcome of blood loss. The extreme degree of these disorders in the body is referred to as post-hemorrhagic shock.

Morphology.

Manifestations of acute anemia are pallor of the skin and anemia of internal organs. Due to a sharp decrease in tissue oxygenation, the production of erythropoietin, which stimulates erythropoiesis, increases. In the bone marrow, there is a significant increase in the number of erythroid cells and the bone marrow acquires a crimson color. In the spleen, lymph nodes, perivascular tissue, foci of extramedullary, or extramedullary, hematopoiesis appear. Normalization of peripheral blood parameters after replenishment of blood loss occurs after about 48-72 hours.

Violation of hemodynamics and a decrease in the intensity of biological oxidation in cells cause the inclusion adaptive mechanisms :

• activation of thrombus formation;
• reactions of cardiovascular compensation for blood loss in the form of narrowing of the lumen of small vessels and ejection of blood from the depot;
• increased cardiac output;
• maintaining the volume of circulating blood due to the flow of fluid from the interstitium into the vessels.

Chronic posthemorrhagic anemia occurs with significant blood loss due to repetitive bleeding, for example, from hemorrhoidal veins, with uterine bleeding, etc. Such blood loss leads to chronic tissue hypoxia and metabolic disorders in them.

Morphology.

Chronic hypoxia contributes to the development of fatty degeneration of parenchymal organs. The yellow bone marrow is transformed into red, as erythropoiesis and myelopoiesis are enhanced. Foci of extramedullary hematopoiesis may appear in the liver, spleen, and lymph nodes. At the same time, with long-term repeated and pronounced co-losses, hypo- and aplasia of the hematopoietic tissue may occur, which indicates the depletion of hematopoiesis.

ANEMIA DUE TO IMPAIRED GENERATION (DEFICIENCY)

These anemias are the result of a lack of a number of substances necessary for normal hematopoiesis - iron, vitamin B 12 , folic acid, etc. Among them, malignant Addison-Birmer anemia is of the greatest importance. which is based on a deficiency of vitamin B 12 and folic acid.

B 12 - deficient, or folic acid deficiency, anemia. The etiology of anemia is associated with a deficiency of vitamin B 12 and folic acid, which regulates normal hematopoiesis in the bone marrow. However, for the activation of folic acid, it is necessary that the vitamin supplied with food B 12 (external factor) combined with the protein formed in the stomach - gastromucoprotein(intrinsic factor), which is produced by additional cells of the glands of the gastric mucosa. Together they form a complex called anti-anemic factor . Then this complex enters the liver and activates folic acid, which, in turn, stimulates erythropoiesis according to the erythroblastic type. If autoimmune gastritis develops and antibodies to additional cells or gastromucoprotein appear, which destroy these cells or the intrinsic factor, then vitamin B 12 is not absorbed in the gastric mucosa and gastromucoprotein is not formed. The same situation occurs with a high resection of the stomach for a tumor or ulcerative process.

Pathogenesis.

As a result of atrophy of the gastric mucosa of an autoimmune nature, a deficiency of folic acid and vitamin B 12 occurs. Erythropoiesis is disturbed and instead of erythrocytes, their precursors are formed - large megaloblasts that appear in the peripheral blood. However, megaloblasts are rapidly destroyed, anemia and general hemosiderosis develop. In addition, with a deficiency of vitamin B 12, the formation of myelin in the sheaths of the nerve trunks is disrupted, which disrupts their function.

Pathological anatomy.

In patients, pallor of the skin, watery blood, petechial hemorrhages are noted, due to atrophy of the mucous membrane of the tongue, it acquires a crimson color ( hunter's glossitis), characterized by atrophic gastritis, thickening and enlargement of the liver due to fatty degeneration and hemosiderosis associated with hypoxia and increased destruction of megaloblasts. In the spinal cord - the collapse of the axial cylinders in the posterior and lateral columns and foci of softening of the brain tissue ( funicular myelosis), which is accompanied by severe neurological symptoms. The bone marrow of flat and tubular bones is red, reminiscent of raspberry jelly. In the spleen and lymph nodes, foci of extramedullary hematopoiesis.

The course of the disease is progressive, with periods of remission and exacerbation. Treatment of anemia with folic acid and vitamin B 12 preparations led to the fact that patients stopped dying from this disease.

ANEMIA DUE TO INCREASED BLEEDING - HEMOLYTIC

These anemias are characterized by the predominance of the process of destruction of erythrocytes (hemolysis) over their formation. The life expectancy of erythrocytes is reduced and does not exceed 90-100 days.

Types of hemolytic anemia

By origin, hemolytic anemia is divided into acquired (secondary) and congenital or hereditary.

Acquired hemolytic anemia can be caused by numerous factors. The etiology of these anemias is associated with the action of physical, chemical and biological factors, including autoimmune, in nature, especially with a deficiency of substances that stabilize erythrocyte membranes, such as α-tocopherol. Of greatest importance are the so-called hemolytic poisons of chemical (compounds of arsenic, lead, phosphorus, etc.) and biological origin. Among the latter are mushroom poisons, various toxic substances formed in the body during severe burns, infectious diseases (for example, malaria, relapsing fever), blood transfusions that are incompatible with the group or Rh factor.

Pathogenesis.

Hemolysis of erythrocytes can occur inside and outside the vessels. At the same time, hemoglobin breaks down and two pigments are synthesized from heme - hemosiderin and bilirubin. Therefore, hemolytic anemia is usually accompanied by the development of general hemosiderosis and jaundice. In addition, erythropenia and hemoglobin breakdown lead to the appearance of severe hypoxia, accompanied by fatty degeneration of parenchymal organs.

Morphology hemolytic anemia is characterized by the development of hyperplastic processes in the bone marrow, in connection with which it acquires a crimson color, the appearance of foci of extramedullary hematopoiesis, severe jaundice of the skin and internal organs, hemosiderosis and fatty degeneration of the liver, heart and kidneys.

Hemolytic disease of the newborn is an example of acquired hemolytic anemia and is of great importance in obstetric and pediatric practice. It is based on the immune conflict between the mother and the fetus on the Rh factor, which has antigenic properties. This factor was first discovered in the erythrocytes of rhesus monkeys and is present in 80-85% of humans. If the mother is Rh-negative, i.e., does not have a Rh factor, and the fetus is Rh-positive, then antibodies against the erythrocytes of the fetus are formed in the mother's body and intravascular hemolysis of erythrocytes occurs in it.

Rice. 43. Sickle cell anemia. Sickle-shaped erythrocytes. electronogram.

In this case, the fetus may die at the 5-7th month of pregnancy, and newborns develop hemolytic anemia, accompanied by anemia and fatty degeneration of internal organs, severe jaundice and hemosiderosis.

Hereditary, or congenital, hemolytic anemias are associated with some genetic defect in the structure of membranes, enzymes, or hemoglobin. This defect is inherited.

Types: congenital hemolytic anemia, depending on the genetic defect, can be caused by membranopathies, fermentopathies, hemoglobinopathies.

Pathogenesis of all congenital hemolytic anemias is basically similar - as a result of one or another genetic defect, either the erythrocyte membrane is destroyed, and the erythrocytes themselves decrease in size and can take on a spherical shape ( microspherocytosis), either the membrane permeability increases and the erythrocytes increase in size due to the influx of an excess amount of fluid, or the synthesis of hemoglobin is disturbed ( hemoglobinosis) and irregularly shaped erythrocytes are formed, containing rapidly decomposing hemoglobin, and retaining oxygen (thalassemia, sickle cell anemia, etc.)(Fig. 43).

Morphology congenital hemolytic anemia differs little from changes in secondary hemolytic anemia, with the exception of the size and shape of red blood cells. Also characteristic are pronounced intravascular hemolysis, hypoxia, hemosiderosis, fatty degeneration of parenchymal organs, hyperplasia of the hematopoietic tissue, foci of extramedullary hematopoiesis, hepato- and splenomegaly are possible.

PATHOLOGY OF THE LEUKOCYTE SYSTEM

The blood of a healthy person at rest on an empty stomach contains 4 10 9 / l of leukocytes. Many leukocytes are found in tissues where they are involved in immune control.

Typical changes in the number of leukocytes per unit volume of blood are characterized by either a decrease - leukopenia, or an increase - leukocytosis, which, as a rule, is a reaction of the leukocyte system that develops in diseases and pathological conditions. Therefore, the cure of the disease leads to the normalization of the leukocyte formula.

Leukopenia is a decrease in the number of leukocytes in a unit of blood volume below normal, usually less than 410 9 /l. It occurs as a result of inhibition of the white germ of the hematopoietic system, with increased destruction of leukocytes, or with the redistribution of blood between the bloodstream and the blood depot, which is observed, for example, in shock.

The value of leukopenia is to weaken the body's defenses and increase its susceptibility to various infectious pathogens.

Types of leukopenia by origin:

• primary leukopenias(congenital or hereditary) are associated with various genetic defects in the hematopoietic system at different stages of leukopoiesis;
• secondary leukopenias arise under the action of various factors on the body - physical (ionizing radiation, etc.), chemical (benzene, insecticides, cytostatics, sulfonamides, barbiturates, etc.), metabolic products or components of various pathogens.

Leukocyte formula- the ratio of different types of circulating leukocytes.

If the number of young forms of neutrophils (stab, metamyelocytes, myelocytes, promyelocytes) located on the left side of the leukocyte formula increases, the formula shifts to the left, which indicates an increase in the proliferation of myelocytic cells. On the right side of the formula are the mature forms of these cells. The cure of the disease leads to the normalization of the leukocyte formula. A decrease in the normal number of leukocytes in the leukocyte formula indicates a decrease in the regenerative capacity of myeloid tissue.

Pathogenesis of leukopenia reflects a violation or inhibition of the process of leukopoiesis, as well as excessive destruction of leukocytes in the circulating blood or in the organs of hematopoiesis, redistribution of leukocytes in the vascular bed, and loss of leukocytes by the body is also possible. At the same time, due to the inhibition of regeneration of leukopoietic tissue at the initial stages of leukopenia, the number of young forms of neutrophils decreases, and an increase in their young forms (i.e., a shift of the leukocyte formula to the left) indicates the cessation of the damaging effect and activation of leukopoiesis. It is also possible the appearance of anisocytosis and poikilocytosis of leukocytes.

Leukocytosis- an increase in the number of leukocytes per unit volume of blood above 4 10 9 /l. It can be physiological, adaptive, pathological, or take the form of a pikemoid reaction.

• Physiological leukocytosis occurs in healthy people in connection with the redistribution of blood during digestion, during physical work.
• adaptive leukocytosis develops in diseases, especially those characterized by inflammation. In this case, the number of leukocytes can increase up to 40 10 9 /l.
• Pathological leukocytosis reflects the tumor nature of leukocytosis and characterizes leukemia.

Leukemoid reaction- an increase in the total number of peripheral blood leukocytes more than 40 10 9 / l with the appearance of their immature forms (promyelocytes, myeloblasts), which makes leukocytosis similar to leukemia.

Types of leukocytosis are associated with an increase in certain forms of leukocytes:

Agranulocytosis- absence or significant decrease in the absolute number of all types of granular granulocytes (leukocytes) - neutrophils, eosinophils, basophils. Agranulocytosis is usually associated with leukopenia.

TUMORS OF THE BLOOD SYSTEM, OR HEMOBLASTOSIS

Hemoblastoses - tumor diseases of the hematopoietic and lymphatic tissue. They are divided into systemic diseases - leukemia , and regional - malignant lymphomas, or hematosarcomas . With leukemia, the bone marrow is primarily affected and tumor cells are found in the blood (leukemia), and with end-stage lymphomas, extensive metastasis occurs with secondary damage to the bone marrow. In terms of prevalence, hemoblastoses occupy the 5th place among all human tumors. In children of the first 5 years of life, they account for 30% of cases of oncological diseases.

Etiology of hemoblastomas does not fundamentally differ from the causes that cause other tumors (see Chapter 10) - these are various mutagenic factors of exo- and endogenous origin that act on stem and semi-stem progenitor cells. Of great importance in the occurrence of hemoblastoses is the hereditary factor.

Pathogenesis.

Many etiological factors affect the genome of stem and semi-stem cells, leading to their malignant transformation. Therefore, the genome is the so-called bottleneck through which mutagens act on proto-oncogenes and anti-oncogenes, turning them into cellular oncogenes, which leads to the appearance of a tumor. The development of hemoblastosis begins with the malignancy of one stem or semi-stem cell, which gives a pool of tumor cells. Consequently, all hemoblastoses are of monoclonal origin, and all subsequent tumor cells develop from the originally mutated cell and belong to the same clone. In addition to malignancy at the level of stem and semi-stem precursor cells, a block of differentiation develops in the pool of tumor cells and they lose their ability to mature.

LEUKOSIS

Leukemia- systemic tumor diseases arising from hematopoietic cells with damage to the bone marrow.

The incidence of leukemia ranges from 3 to 10 per 100,000 population. Men get sick 1.5 times more often than women. Acute leukemias are more common between the ages of 10 and 18, while chronic leukemias are more common in people over 40 years of age.

Morphogenesis.

In leukemia, the tumor tissue initially grows in the bone marrow and gradually suppresses and displaces normal hematopoietic sprouts. Therefore, patients with leukemia develop anemia, platelet-, lymphocyte-, granulocytopenia, which leads to increased bleeding, hemorrhages, decreased immunity and the addition of infectious diseases. Metastasis in leukemia is the appearance of leukemic infiltrates in the liver, spleen, lymph nodes, vascular walls, etc. Vessel obstruction by tumor cells leads to the development of organ infarcts and ulcerative necrotic complications.

Leukemia classification based on 5 signs of these diseases.

1. According to the degree of differentiation of tumor cells allocate undifferentiated, imperious and cytic leukemias. At a high level of differentiation block, tumor cells resemble undifferentiated and blast forms of hemopoiesis. Such leukemias are acute and very malignant.
   When differentiation stops at the level of procytic and cytic precursor cells, leukemias proceed chronically and are less malignant.
2. According to the cytogenetic acute leukemias are subdivided into lymphoblastic, myeloblastic, monoblastic, erythromyeloblastic, megakaryoblastic, undifferentiated. Chronic leukemias are divided into leukemias of myelocytic origin (chronic myelocytic, chronic neutrophilic, chronic eosinophilic, etc.), lymphocytic (chronic lymphocytic leukemia and paraproteinemic leukemias - myeloma, primary macroglobulinemia of Waldenström, etc.) and monocytic - chronic monocytic leukemia, histiocytosis X.
3. By immune phenotype tumor cells: based on the detection of markers of their antigens.
4. According to the total number of leukocytes in peripheral blood allocate leukemias.
   • leukemic- tens and hundreds of thousands of leukocytes in 1 µl of blood, including blasts;
   • subleukemic- the number of blood leukocytes is 25-50 10 9 /l, including blast forms;
   • leukopenic- the number of leukocytes in the peripheral blood is below normal, but there are blasts;
   • aleukemic- the number of leukocytes "in the blood is less than normal and there are no blast forms.
5. According to the nature of the flow, there are:
   1. acute leukemias (they are also undifferentiated and blast);
   2. chronic leukemia (cytic).

Acute leukemias develop from all sprouts of morphologically undifferentiated hematopoietic progenitor cells. The duration of the course of the disease is 2-18 months, with successful treatment, remissions can last up to 5-8 years.

Morphogenesis.

Various forms of acute leukemia have stereotyped morphological manifestations. They are conjured in the development of leukemic infiltration of the bone marrow by atypical cells of the early stages of hematopoiesis (Fig. 44). Due to the non-differentiation of these cells, their cytogenetic affiliation can only be identified using cytochemical and immunohistochemical methods. The bone marrow of tubular bones becomes red, with some acute leukemias it acquires a greenish color, characteristic of pus, - pyoid bone marrow. In this case, normal cells of hematopoiesis are replaced by tumor cells. In the peripheral blood and in the bone marrow, there are only blast and mature forms of cells, but their intermediate forms are absent. This pattern of blood is called leukemic failure ". Leukemic infiltrates are found in the lymph nodes, spleen and liver, which leads to an increase in inflammation of the oral cavity and tonsil tissue, which is complicated by necrotizing gingivitis, tonsillitis, necrotic tonsillitis, and leukemic meningitis develops with infiltration of the meninges. Suppression of the erythrocyte germ leads to increasing hypoxia and fatty degeneration of parenchymal organs.

Rice. 44. Bone marrow in acute lymphoblastic leukemia. The brain tissue consists mainly of lymphoblasts (a), the lumen of the vessels is filled with the same cells (b).

As a result of thrombocytopenia, damage to the liver and vessel walls, patients develop hemorrhagic syndrome up to cerebral hemorrhages and fatal gastrointestinal bleeding. Against this background, sepsis sometimes joins, leading patients to death (Fig. 45).

Most common, especially in children, acute lymphoblastic leukemia, associated with tumor transformation of T- and B-lymphocyte progenitors, and acute myeloid leukemia, which adults suffer more often, due to tumor proliferation of myeloid progenitor cells.

Rice. 45. Acute leukemia, a - leukemic infiltration of the liver (shown by arrows); b - tonsil necrosis (necrotic tonsillitis); c - leukemic infiltration of the kidneys; d - multiple hemorrhages in the epicardium and endocardium; e - leukemic infiltration of the bone marrow (pyoid bone marrow), thinning of the cortical layer of the femur (shown by an arrow).

Rice. 46. The liver in chronic myeloid leukemia. Growth of myeloid cells (a) along the sinusoids.

Chronic leukemia flow for more than 4 years, with successful treatment, remission of the disease can last 20 years or more. Chronic leukemias differ from acute ones by cytic differentiation of tumor cells and a longer course, which has certain stages:

• the monoclonal stage is characterized by the presence of only one clone of tumor cells, flows for years, is relatively benign;
• polyclonal stage, or power crisis , is associated with the appearance of secondary tumor clones, is characterized by a rapid malignant course, and 80% of patients die at this stage.

Morphogenesis.

Leukemic infiltrates grow in the bone marrow, liver, spleen, kidneys, lymph nodes, intestinal mesentery, often in the mediastinum, and therefore these organs and tissues increase sharply in size and can compress neighboring organs (Fig. 46). Splenomegaly (the weight of the spleen reaches 6-8 kg) and hepatomegaly (the weight of the liver is 5-6 kg) are especially pronounced. Leukemia thrombi are formed in the vessels, which can lead to the development of ischemic heart attacks, more often in the spleen and kidneys. In the blood, the number of neutrophilic leukocytes or lymphocytes increases, there are many transitional cellular forms. Anemia, thrombocytopenia, significant immunosuppression and a predisposition to infectious complications are pronounced, from which patients often die. The bone marrow is gray-red. Fatty degeneration of parenchymal organs gives them a gray-yellow color.

The benign course is replaced by a blast crisis. At the same time, the number of blast forms in the blood rapidly increases - myelo-, erythro-, lympho-, megakaryoblasts, etc. The total number of peripheral blood leukocytes can reach several million in 1 μl. Power crisis is the cause of death of patients.

PARAPROTEINEMIC LEUKEMIA

Paraproteinemic leukemias are characterized by the ability of tumor cells to synthesize homogeneous immunoglobulins or their fragments - paraproteins. At the same time, tumor cells are atypical plasmocytes and therefore retain the ability to synthesize atypical immunoglobulins in a perverted form.

Myeloma (plasmocytoma)- chronic leukemia, the most common among paraproteinemic hemoblastoses.

It occurs mainly in adults and with modern methods of treatment can last 4-5 years. The basis of the disease is a tumor growth in the bone marrow of atypical plasma cells, called myeloma cells. They synthesize paraproteins that are found in the blood and urine of patients. According to the nature and prevalence of the tumor infiltrate in the bone marrow, nodular and diffuse forms of the disease are distinguished.

With a nodular form, plasmacytoma forms tumor nodes in the bone marrow, usually flat bones (cranial vault, ribs, pelvis) and vertebrae. Leukemic infiltration is accompanied by liquefaction of the bone or its axillary resorption (osteolysis and osteoporosis) with the formation of the correct form of rounded defects, which on the radiograph look like smooth-walled holes. Sinus resorption causes the release of calcium from the bones and the development of hypercalcemia with the appearance of multiple calcareous metastases in the muscles and parenchymal organs. In addition, pathological fractures of the bones occur.

With a generalized form of multiple myeloma proliferation of myeloma cells occurs, in addition to the bone marrow, in the spleen, lymph nodes, liver, kidneys and other internal organs.

Morphogenesis.

Abnormal immune proteins (paraproteins) are found in the peripheral blood, including the finely dispersed Bence-Jones protein, which easily passes through the kidney filter and is detected in the urine. Due to the high concentration of Bence-Jones protein, paraproteinemic nephrosis develops. In addition, due to disturbances in the normal synthesis of immunoproteins, plasmacytoma is often complicated by the development of amyloidosis with kidney damage. Therefore, the cause of death of these patients is often uremia. Due to the sharp suppression of the function of the immune system, a secondary infection can join the underlying disease, which also causes death in patients with multiple myeloma.

MALIGNANT LYMPHOMAS (HEMATOSARCOMAS)

Malignant lymphomas (hematosarcomas)- regional malignant tumors of lymphoid tissue, having a monoclonal origin.

Lymphomas develop from immature forms of lymphocytes and affect the lymphatic tissue of any one area, however, in the terminal stage of the disease, generalization of the tumor process with the development of metastases to the bone marrow is possible.

Etiology.

The causes of malignant lymphomas in principle do not differ from the causes of tumors of other origin. However, it has been proven that some of the lymphomas as well as some other leukemias, is of viral origin. Hereditary predisposition to the disease is not excluded. The transformation of normal hematopoietic cells into tumor cells occurs as a result of changes in the genome, as a result of which the normal genetic program of hematopoiesis changes in the direction of tumor atypism.

Classification of lymphomas.

1. According to clinical and morphological features:
   • lymphogranulomatosis, or Hodgkin's disease;
   • non-Hodgkin's lymphomas.
2. According to the source of growth (cytogenesis):
   • B-lymphocytic;
   • T-lymphocytic.
3. According to the degree of differentiation of tumor cells:
   • low malignancy;
   • moderate malignancy;
   • high malignancy.

Lymphogranulomatosis (Hodgkin's disease) described in 1832 by the English physician T. Hodgkin. The frequency of the disease is 3 cases per 100,000 population, or 1% of all malignant neoplasms. The tumor affects the lymph nodes, usually in one area - cervical, mediastinal, retroperitoneal, less often axillary or inguinal.

Morphogenesis.

Affected lymph nodes increase in size, merge with each other and form large packets. At the beginning of the disease, the lymph nodes are soft, pink on the cut. As the lymphoma progresses, necrotic and then sclerotic changes develop in them, in connection with which the lymph nodes thicken, look dry and variegated on the cut. In its development, lymphogranulomatosis goes through several stages - from an isolated lesion of a group of lymph nodes to a generalized lesion of internal organs with suppression of lymphoid tissue and its replacement with sclerosis fields.

On microscopic examination, the tumor consists of polymorphic tumor cells of the lymphocytic series, among which there are characteristic giant cells with a lobed nucleus and a narrow rim of the cytoplasm - Berezovsky-Sternberg cells. These cells serve as a diagnostic sign of Hodgkin's disease. In addition, characteristic Hodgkin cells - large cells with a large light nucleus and a dark nucleolus.

Often at the end of the disease, it becomes generalized with damage to many internal organs - the stomach, lungs, liver, and skin. At autopsy of those who died from lymphogranulomatosis, the spleen looks especially demonstratively - it is enlarged, dense, red in section with multiple white-yellow foci of necrosis and sclerosis, which makes it look like a special type of granite - porphyry(porphyritic spleen).

Non-Hodgkin's lymphomas.

This is a group of malignant tumors from undifferentiated and blast forms of B- and T-cells of lymphatic tissue. The diagnosis of these diseases requires a mandatory morphological and immunohistochemical study of biopsy specimens of lymph nodes.

The hematopoietic organs include the liver, bone marrow, lymph nodes, and spleen. Blood cells are produced here: red blood cells (erythrocytes) - mainly in the red bone marrow, white blood cells (leukocytes) - in the spleen and lymph nodes (one of the forms of leukocytes is produced in the spleen - monocytes, in the lymph nodes - lymphocytes), blood platelets (platelets) - in the red bone marrow. Among the diseases of the hematopoietic organs, anemia, or anemia, is the most common. Blood delivers nutrients to all cells of the body and removes harmful products. It consists of a transparent liquid - plasma, in which the shaped elements are located. The total amount of blood in a healthy person's body is about 5 liters. Blood plasma is water with protein substances dissolved in it, sugar, the smallest particles of fat, various salts, and oxygen (in a small amount).

Diseases of the blood, hematopoietic organs.

Diseases of the blood, hematopoietic organs are pathological conditions characterized by a violation of the quantitative and qualitative composition of blood elements, as well as a complete or partial decrease in the functions of the circulatory system.

The hematopoietic system is one of the most important in the human body. Its main task is to regulate all vital signs of the human body.

Since ancient times, there have been many legends associated with the unusual and magical properties of blood. That is why the origin of most diseases since ancient times, people considered associated with the presence of "bad" or "good" blood in the patient's body.

Blood diseases: varieties of diseases that currently exist.

In modern medical practice, the division of the pathology of the circulatory system into 4 groups is used, corresponding to each of the blood cells. So:

1. Damage to erythrocytes.
2. Platelet damage.
3. Leukocyte injury.
4. Violation in the plasma composition of the blood.

So, let's analyze each of the above pathologies.

Diseases of the blood, hematopoietic organs. anemia.

Anemia is a pathological condition of the body, characterized by the fact that there is a decrease in the total number of red blood cells or hemoglobin in a volumetric unit of the patient's blood. With some pathology, not only quantitative, but also qualitative changes in the structural state of red blood cells are noted.

Causes leading to the development of anemia:

1. Extensive blood loss after injuries and surgical interventions.
2. Anemia due to circulatory disorders.

A. Anemia caused by iron deficiency in the blood.

B. Anemia sideroachrestic, iron-saturated.

B. Anemia caused by vitamin B-12 deficiency, "pernicious". The lack of vitamins can be, both due to their small intake, and due to their increased decay or low absorption.

D. Hypoaplastic anemias. They develop both due to external causes and due to impaired functional ability of the body caused by aplastic processes in the bone marrow.

D. Anemia B-12 folic-"ahrestic".

E. Metaplastic anemias.

1. Anemia that develops due to the fact that the destruction of blood cells occurs too quickly. These are the so-called hemolytic anemias. Destruction occurs due to the fact that the body is affected by both external and internal causes. These include hemoglobino- and erythrocytopathies, enzymopathies.

Clinical manifestations of anemia.

All anemias have manifestations that are present in any case. This is dizziness, headache, nausea, weakness, sweating, palpitations, changes in taste preferences, to the point that the patient begins to eat inedible food. On examination, the doctor notes the pallor of the skin, possibly a sharp weight loss. Loss of consciousness for a short time is possible. In addition, there are violations of the digestive system.

Diagnostic methods aimed at establishing this pathology.

1. Clinical and biochemical blood tests.
2. Esophagofibrogastroduodenoscopy.
3. If necessary, perform magnetic resonance and computed tomography.

Anemia treatment.

It is aimed at correcting those violations, as a result of which this disease has developed. These can be both blood substitutes that are administered intravenously, and drugs containing iron in its composition in its various manifestations. In addition, vitamins, drugs that act on the gastrointestinal tract are prescribed. In the presence of urgent surgical pathology, surgical treatment is performed.

Diseases of the blood, hematopoietic organs. Agranulocytosis.

Agranulocytosis is a pathological condition of the body that develops in many diseases. It is characterized by the fact that there is a decrease or complete disappearance of such blood elements as granulocytes. If we divide this pathology by age groups and gender, then more often adults over forty years of age suffer from this pathology, female patients predominate among them.

By classification allocate congenital and acquired agranulocytosis. In addition, if classified according to the mechanism of development of pathology, there are:

1. Agranulocytosis of unknown etiology.
2. Agranulocytosis, which develops as a result of immune processes in the patient's body.
3. Immune hapten agranulocytosis.
4. Myelotoxic agranulocytosis.

According to clinical manifestations and course, acute and chronic agranulocytosis is distinguished, which can occur in mild, moderate or severe forms.

Reasons for the development of agranulocytosis.

1. The impact of ionizing treatment, medicinal or cytostatic drugs on the patient's body.
2. Autoimmune processes in the patient's body.
3. The presence of an infectious disease.
4. Genetic damage and hereditary predisposition.
5. The presence of oncological pathology.

Symptoms accompanying agranulocytosis.

1. A sharp increase in body temperature.
2. Sweating, weakness, dizziness, sweating, the appearance of pain in the joints.
3. The presence of ulcers on the mucous membrane of the oral cavity.
4. Excessive fragility of the vascular wall, expressed in the appearance of multiple bruises, bruises, nosebleeds. Sometimes clinical manifestations can progress, and then it is possible to excrete blood in the urine and feces. In severe cases, DIC develops.
5. Lymph nodes increase, in addition, there is an increase in the liver, spleen.
6. Symptoms of an "acute abdomen" are possible. Pain, diarrhea, tension in the muscles of the anterior abdominal wall. In addition, a secondary infection begins to join.

Diagnostic measures aimed at recognizing agranulocytosis.

1. Blood test (biochemical and clinical).
2. Ultrasound examination of the abdominal organs.
3. X-ray of the chest organs.
4. Puncture and examination of the bone marrow.
5. Consultation of related specialists.

Treatment of agranulocytosis.

1. Treatment is strictly in a hospital for hematological patients.
2. Compliance with the rules of asepsis and antisepsis to prevent the occurrence of a secondary infection.
3. Stopping medications that could cause this pathology.
4. Appointment by the attending physician of antibiotics, drugs aimed at preventing the development of a fungal infection.
5. Transfusion of blood and its components.
6. The introduction of immunoglobulins and sera.
7. Reception of glucocorticosteroids.
8. Carrying out, if necessary, plasmapheresis.

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