An example of a blood disease caused by changes in the structure and functions of cellular elements is sickle cell anemia, “lazy white blood cell” syndrome, etc.

Analysis of urine. It is carried out to identify concomitant pathology (diseases). Bloodletting is performed to normalize the number of blood cells and reduce its viscosity. Before bloodletting, medications are prescribed to improve blood flow and reduce blood clotting.

An excess number of red blood cells appears in the blood, but the number of platelets and neutrophilic leukocytes also increases (to a lesser extent). The clinical manifestations of the disease are dominated by manifestations of plethora (plethora) and complications associated with vascular thrombosis. The tongue and lips are bluish-red, the eyes seem to be bloodshot (the conjunctiva of the eyes is hyperemic). This is due to excessive blood supply and the participation of the hepatolienal system in the myeloproliferative process.

Patients have a tendency to form blood clots due to increased blood viscosity, thrombocytosis and changes in the vascular wall. Along with increased coagulability blood and thrombus formation in polycythemia, bleeding from the gums and dilated veins of the esophagus is observed. The treatment is based on reducing blood viscosity and combating complications - blood clots and bleeding.

Bloodletting reduces blood volume and normalizes hematocrit. The outcome of the disease can be the development of myelofibrosis and cirrhosis of the liver, and with progressive anemia of the hypoplastic type - transformation of the disease into chronic myeloblastic leukemia.

Typical examples of blood diseases caused by changes in the number of cellular elements are, for example, anemia or erythremia (increased number of red blood cells in the blood).

Blood disease syndromes

Diseases of the blood system and blood diseases are different names for the same set of pathologies. But blood diseases are a pathology of its immediate components, such as red blood cells, leukocytes, platelets or plasma.

Hemorrhagic diathesis or pathology of the hemostasis system (blood clotting disorders); 3. Other blood diseases (diseases that do not relate to hemorrhagic diathesis, anemia, or hemoblastosis). This classification is very general, dividing all blood diseases into groups based on which general pathological process is leading and which cells are affected by the changes.

The second most common anemia associated with impaired synthesis of hemoglobin and red blood cells is the form that develops in severe chronic diseases. Hemolytic anemia, caused by increased breakdown of red blood cells, is divided into hereditary and acquired.

Anemic syndrome

II A – polycythemic (that is, with an increase in the number of all blood cells) stage. A general blood test reveals an increase in the number of red blood cells, platelets (blood platelets), and leukocytes (except lymphocytes). Palpation (palpation) and percussion (tapping) reveal an enlarged liver and spleen.

The number of leukocytes (white blood cells, normal 4-9x109 g/l) can be increased, normal or decreased. The number of platelets (blood platelets, the gluing of which ensures blood clotting) initially remains normal, then increases and decreases again (the norm is 150-400x109 g/l). Erythrocyte sedimentation rate (ESR, nonspecific laboratory value, reflecting the ratio of blood protein species) usually decreases. Ultrasound examination (ultrasound) of internal organs evaluates the size of the liver and spleen, their structure for damage by tumor cells and the presence of hemorrhages.

OBJECTIVE OF THE LESSON: to teach to identify the main syndromes in diseases of the blood system; study etiopathogenesis, symptomatology, basic principles for diagnosing certain diseases of the hematopoietic system.

THE STUDENT SHOULD KNOW:

1. anemia syndrome;

2. myeloproliferative syndrome;

3. lymphoproliferative syndrome;

4. hemorrhagic syndrome;

5. myelotoxic syndrome;

6. bone marrow failure syndrome;

7. definition of the concept of “acute posthemorrhagic anemia”, “chronic posthemorrhagic anemia”, “megaloblastic anemia”, “acute leukemia”, “chronic myeloid leukemia”, “chronic lymphocytic leukemia”, an idea of ​​their etiology and pathogenesis;

8. classification of anemia;

9. syndromes that make up the clinical picture of acute posthemorrhagic, chronic iron deficiency and megaloblastic anemia;

10. mechanism of symptoms in anemia and leukemia;

11. classification of leukemia;

12. syndromes that make up the clinical picture of acute leukemia, chronic myelo- and chronic lymphocytic leukemia;

13. the most informative laboratory and instrumental research methods for anemia and leukemia;

14. modern principles of treatment of anemia and leukemia.

THE STUDENT SHOULD BE ABLE TO:

1. conduct a physical examination of patients with blood diseases;

2. identify the main clinical syndromes for blood diseases;

3. make a plan for additional benefits informative research for anemia and leukemia;

4. interpret the results of paraclinical research methods;

5. based on the identified syndromes, formulate a diagnosis.

THE STUDENT MUST HAVE PRACTICAL SKILLS

1. examination of a patient with anemia and drawing up a conclusion;

2. examination of a patient with leukemia and drawing up a conclusion;

3. prescribing additional research methods for these diseases;

4. formulation of the diagnosis.

1. ANEMIC SYNDROME

Anemic syndrome is observed in hematological and other diseases.

It is caused by a decrease in hemoglobin content per unit volume of blood (often with a simultaneous decrease in the content of red blood cells), insufficient oxygen supply to tissues and is represented by nonspecific symptoms.

The mechanism of majority emergence clinical signs- hypoxia of organs and tissues. With a gradual increase in anemia, compensatory mechanisms are activated, which can delay the onset of symptoms in the patient.

1.1. Complaints

Associated with hypohemoglobinemia and tissue hypoxia, they do not always correspond to the level of hemoglobin due to adaptation of the patient’s body. Include:

- general weakness fast fatiguability and decreased performance;

– dizziness, tinnitus, flickering of spots before the eyes;

– shortness of breath on exertion, predominantly inspiratory;

– heartbeat;

– stabbing pains in the region of the heart;

– fainting states;

– loss of appetite;

– flashing “flies” before the eyes;

– persistent headaches; memory loss, drowsiness.

1.2. Physical research

External research:

Pallor skin and mucous membranes:

1. with an alabaster or greenish tint (iron deficiency anemia);

2. with icterus (hemolytic anemia);

– dull, brittle hair and nails;

Pastiness of the lower extremities, face.

Respiratory system:

Increased breathing is compensatory.

Cardiovascular system (myocardial dystrophy syndrome):

Tachycardia, often arrhythmia;

Unsharp shift of the borders of relative dullness of the heart to the left;

Deafness of heart sounds;

Systolic murmur over all points of auscultation of the heart and a “spinning top” murmur over large veins (acceleration of blood flow and decrease in blood viscosity);

Moderate decrease in blood pressure;

Digestive system:

Atrophy of the mucous membrane and papillae of the tongue;

Bright red tongue (B 12 deficiency anemia);

Paleness of the mucous membranes;

Enlarged liver and spleen (with increased destruction of red blood cells).

1.3. Paraclinical data

UAC: decrease in Hb and red blood cell counts. Changes in the number of reticulocytes, color index, hematocrit, erythrocyte volume - depending on the cause of the anemic syndrome.

2. MYELOPROLIFERATIVE SYNDROME

A condition characterized by absolute neutrophilia with a shift in leukocyte formula to myelocytes and/or blasts:

Hepatosplenomegaly;

Increase lymph nodes, rarely;

In the bone marrow - proliferation of granulocytic cells.

3. LYMPHOPROLIFERATIVE SYNDROME

Condition characterized by absolute lymphocytosis:

Enlargement of all groups of lymph nodes;

Hepatomegaly, often splenomegaly;

Skin manifestations (hyperemic papules with thickening in the center, itching - in the terminal stage);

In the bone marrow – an increase in the percentage of lymphocytes.

4. HEMORRHAGIC SYNDROME

Hemorrhagic syndrome - pathological bleeding, characterized by internal and external bleeding, the occurrence of hemorrhages.

Highlight five types of bleeding(Barkagan Z.S., 1975):

1. Hematoma(massive, deep, intense and painful hemorrhages in the muscles, large joints, subcutaneous and retroperitoneal tissue, serous membranes), observed in hemophilia, overdose of anticoagulants.

2. Petechial spotted or microcirculatory: petechiae (small, pinhead-sized hemorrhages that occur with minimal bruises, or spontaneously) and ecchymoses (bruises as a result of blood soaking the skin and mucous membranes) occur with quantitative and qualitative platelet defects; This pathology is also characterized by hemorrhages on the mucous membranes and bleeding (uterine, kidney, nasal, after surgical interventions).

3. Mixed, with a predominance of the microcirculatory type - in DIC syndrome.

4. Vascular purple(rashes mainly on lower limbs, symmetrical, bright red; easily caused or spontaneous bleeding from the mucous membranes) due to damage to the vascular endothelium in hemorrhagic vasculitis, periarteritis nodosa, infectious diseases, the effects of drugs.

5. Angiomatous(bleeding, very persistent):

Subcutaneous and submucosal hemorrhages;

Hemarthrosis;

Intermuscular hematomas;

Nasal, pulmonary, gastrointestinal, uterine bleeding;

Bleeding of wounds;

Telangiectasia;

Increased bleeding time according to Duke (in case of disruption of the platelet component of hemostasis);

Reduced platelet count (with thrombocytopenic purpura);

Signs of anemia, leukemia in a general blood test;

Determination of prothrombin time (PT) and activated partial thromboplastin time (APTT):

PT is prolonged, APTT is not changed (factor VII deficiency - hypoconvertinemia);

PT is not changed, APTT is prolonged (factor VIII, IX deficiency);

PT and APTT are prolonged (factor X, V, II, I deficiency);

Decrease in fibrinogen in blood plasma.

5. MYELOTOXIC SYNDROME

A condition characterized by pancytopenia that has developed due to the use of cytostatics:

Thrombocytopenia;

Agranulocytosis.

6. BONE BRAIN FAILURE SYNDROME

A condition characterized by cytopenia due to inhibition of normal hematopoiesis by a pathological clone of cells (blasts in acute leukemia).

Anemia– a condition characterized by a decrease in the hemoglobin content per unit volume of blood (often with a simultaneous decrease in the number of red blood cells), accompanying both hematological diseases themselves and many other diseases.

7.1. Classification of anemia

(Butler P.A., Vorobyov A.I., 1994)

I. Anemia due to blood loss (post-hemorrhagic):

Acute posthemorrhagic anemia;

Chronic posthemorrhagic anemia.

II. Anemia due to impaired formation of red blood cells and hemoglobin:

1. Iron-deficiency anemia;

2. iron redistribution anemia (impaired iron reutilization);

3. iron-saturated (sideroachrestic) anemia associated with impaired heme synthesis;

4. megaloblastic anemia associated with impaired DNA synthesis:

In 12 - and folate deficiency anemia;

Anemia caused by hereditary deficiency of enzymes involved in the synthesis of purine and pyrimidine bases;

At 12 - achrestic anemia;

5. hypoproliferative anemia;

6. anemia associated with bone marrow failure:

Hypoplastic (aplastic) anemia;

Refractory anemia in myelodysplastic syndrome;

7. metaplastic anemia:

Anemia in hemoblastoses;

Anemia due to cancer metastases to the bone marrow;

8. dyserythropoietic anemia.

III. Anemia due to increased blood destruction (hemolytic).

1. Hereditary:

Associated with a violation of the structure of the erythrocyte membrane;

Associated with enzyme deficiency in red blood cells;

Associated with impaired hemoglobin synthesis.

2. Purchased:

Autoimmune;

Paroxysmal nocturnal hemoglobinuria;

Medicinal;

Traumatic and microangiopathic;

Due to poisoning with hemolytic poisons and bacterial toxins.

IV. Mixed anemia.

Anemia is classified according to the level of decrease in hemoglobin:

1) Mild degree(90-120 g/l).

2) Moderate severity (70-90 g/l).

3) Severe degree (less than 70 g/l, in old age severe anemia with Hb 75 g/l).

7.2. Acute posthemorrhagic anemia

This is anemia caused by rapid and massive blood loss.

7.2.1. Etiology

Acute posthemorrhagic anemia occurs as a result of profuse bleeding with:

Injuries and wounds accompanied by damage blood vessels;

Lung diseases (cancer, tuberculosis, abscess, bronchiectasis, isolated pulmonary hemosiderosis);

Diseases gastrointestinal tract(peptic ulcer of the stomach and duodenum, cancer of the stomach, intestines, varicose veins of the esophagus and rectum with cirrhosis of the liver);

Diseases of the urinary system (cancer, hemorrhagic cystitis, etc.);

Diseases of the genitals (uterine cancer, fibroids, etc.);

Diseases of the blood system (hemophilia, hemorrhagic diathesis).

7.2.2. Pathogenesis

Acute blood loss® acutely developed decrease in stroke volume of blood due to loss of plasma part and red blood cells ® decrease in the volume of circulating red blood cells ® acute hypoxia ® appearance of shortness of breath, palpitations ® increase in erythropoietin content ® proliferation of erythropoietin-sensitive cells ® increase in the percentage of erythrokaryocytes ® release of reticulocytes.

7.2.3. Clinical picture

For posthemorrhagic anemia The following syndromes are characteristic:

Ongoing bleeding(external bleeding, pulmonary hemorrhage, bloody vomiting, bleeding from the rectum, black tarry stools).

Anemia syndrome:

General weakness, dizziness, tinnitus, flickering of spots before the eyes;

Fainting;

Sharp pallor of the skin and visible mucous membranes;

Sticky cold sweat;

Decrease in temperature;

Shallow, rapid breathing;

Decreased blood pressure;

Tachycardia;

Rapid, soft or thready pulse;

Muffling of heart sounds;

Systolic murmur at the apex.

The severity of the condition is determined by the amount of blood lost and the rate of blood loss.

7.2.4. Paraclinical data

- early period(reflex vascular phase of compensation) - red blood counts do not change (the first hours after blood loss, duration up to 1.5 days), rarely reduced;

- second period(hydremic phase of compensation) - the amount of hemoglobin and red blood cells is reduced, the color indicator is not changed, lasts 4-5 days;

- third period(bone marrow phase of compensation) – the content of reticulocytes, polychromatophils is increased, the appearance of normocytes, gradual normalization of the number of erythrocytes, hemoglobin is slightly reduced (bone marrow phase);

Reduced platelet count (high consumption during bleeding).

7.2.5. Modern principles of treatment

1. Stop bleeding.

2. Anti-shock measures (albumin, electrolyte solutions, colloidal solutions).

3. Fresh frozen plasma.

4. Red blood cell mass (30% of lost blood is injected) - after stopping bleeding with signs of hypoxia.

5. Cardiotonic, vasoactive drugs.

7.3. Chronic iron deficiency anemia

This is anemia caused by iron deficiency in the blood serum, bone marrow and depot. Latent iron deficiency is characterized by a decrease in the amount of iron in its depot and a decrease in the level of transport iron in the blood, while hemoglobin and red blood cells are still normal. Iron deficiency anemia is characterized by a decrease in all metabolic iron reserves, including transport, and a decrease in the number of red blood cells and hemoglobin.

7.3.1. Etiology

Occurs when:

1. Chronic blood loss:

Respiratory system (tuberculosis, lung cancer with decay, bronchiectasis);

Gastrointestinal tract ( varicose veins esophageal veins, erosive esophagitis, diaphragmatic hernia, peptic ulcer of the stomach and intestines, cancer of the stomach and intestines with decay, polyposis of the stomach and intestines, hemorrhoids, hookworm);

Urinary system (renal tumor, hematuria due to glomerulonephritis, urolithiasis, bladder cancer, renal tuberculosis);

Genitals (long and heavy menstruation, endometriosis, uterine fibroids, malignant tumors uterus, dysfunctional uterine bleeding);

Goodpasture's syndrome;

Hemosiderosis;

Nosebleeds in patients with hemorrhagic diathesis, hypertension;

Iatrogenic blood loss (donation, hemodialysis);

- “hysterical bleeding” (Lastaney de Ferjoles syndrome) – artificially induced bleeding in persons with psychopathic disorders.

2. Increased need for iron:

Pregnancy, childbirth, lactation;

Period of puberty and growth;

Intense sports;

At 12 - deficiency anemia during treatment.

3. Impaired absorption of iron:

Malabsorption syndrome;

Small bowel resection;

Gastric resection.

4. Impaired intake of iron from food:

Vegetarianism;

Low socio-economic standard of living;

Frequent consumption of strong tea, which reduces the absorption of iron in the small intestine.

5. Impaired iron transport:

Congenital hypo- and atransferrinemia;

Hypoproteinemia of various origins;

The appearance of antibodies to transferrin and its receptors.

7.3.2. Pathogenesis

Chronic bleeding® iron deficiency ®:

2. ® decrease in heme synthesis ® decrease in the formation of hemoglobin, globin, protoporphyrin ® tissue hypoxia;

2. ® reduction in the synthesis of iron-containing enzymes ® damage to epithelial tissues (atrophy of the mucous membrane of the digestive tract; trophic changes in the skin and its derivatives).

7.3.3. Clinical picture

Consists of the following syndromes:

1. Anemic syndrome:

Weakness, increased fatigue, decreased performance;

Noise in the ears, dizziness, flickering of spots before the eyes;

Palpitations, shortness of breath on exertion;

Fainting conditions;

Paleness of the skin and visible mucous membranes;

Tachycardia, arrhythmia, expansion of the borders of the heart to the left, dullness of heart sounds, systolic murmur (myocardial dystrophy syndrome);

Arterial hypotension.

2. Sideropenic syndrome(caused by tissue iron deficiency, which leads to a decrease in the activity of many enzymes - cytochrome oxidase, peroxidase, etc.) :

Perversion of taste;

Addiction to spicy, salty, sour, spicy foods;

Perversion of smell;

Severe muscle weakness and fatigue, muscle atrophy and decreased muscle strength;

Dystrophic changes in the skin and its appendages (dryness, peeling, cracking, dullness, fragility, loss, early graying of hair and nails, symptom of koilonychia - spoon-shaped concavity of nails);

Angular stomatitis – cracks, “jams” in the corners of the mouth;

Glossitis – a feeling of pain and swelling in the tongue, redness of its tip (“varnished” tongue), a tendency to periodontal disease and caries;

Atrophy of the gastrointestinal mucosa - dry mucosa, pain when swallowing, atrophic gastritis and enteritis;

Symptom of “blue sclera”;

Imperative urge to urinate, inability to hold urine when laughing or sneezing due to weakness of the sphincters;

- “sideropenic subfebrile condition”;

Pronounced predisposition to acute respiratory viral infections, chronic infections;

Reduced reparative processes in the skin and mucous membranes.

7.3.4. Diagnostics

Decrease in the number of red blood cells;

Decreased hemoglobin (lower limit of normal for men - 130 g/l, for women - 120 g/l);

Reduction in color index less than 0.85;

Hypochromia of erythrocytes;

Anisocytosis, poikilocytosis, microcytosis;

The content of reticulocytes is normal, an increase is possible after treatment;

Tendency to leukopenia;

A moderate increase in ESR is possible.

2. Serum iron and ferritin are reduced.

3. Bone marrow examination - reduction in the number of sideroblasts (usually not carried out, since the genesis is known).

4. Examination of stool occult blood(clarification of the genesis of anemia).

5. ECG: decreased amplitude, negative T waves in the precordial leads.

6. FGDS (clarification of the genesis of anemia, mucosal atrophy).

7. FCS, irrigoscopy (clarification of the genesis of anemia, mucosal atrophy).

8. X-ray of the lungs (clarification of the genesis of anemia, mucosal atrophy).

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

Causes of damage to the blood system.

Anemia.

Among the most common reasons that cause anemia, the following are important:

• acute blood loss (trauma);
• chronic blood loss various localizations(gastrointestinal, uterine, nasal, renal) due to various diseases;
• disturbances in the intestinal absorption of iron that comes with food (enteritis, intestinal resection);
• increased need for iron (pregnancy, lactation, rapid growth);
• common dietary iron deficiency (malnutrition, anorexia, vegetarianism);
• vitamin B12 deficiency (insufficient intake from food - 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 tapeworm infestation) ;
• impaired absorption of folic acid; bone marrow diseases; various hereditary causes.

Leukemia.

The reasons have not been fully elucidated, but what is known is that they may be hereditary predisposition, ionizing radiation, chemicals (varnishes, paints, pesticides, benzene), viruses. Damage to the hemostatic system is most often caused by hereditary factors.

Symptoms of blood diseases.

Often patients with blood diseases complain of weakness, easy fatigue, dizziness, shortness of breath during exercise, interruptions in heart function, loss of appetite, and decreased performance. These complaints are usually manifestations of various anemias. In case of acute and heavy bleeding suddenly appear severe weakness, dizziness, fainting.

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

Patients also often complain of itchy skin.

With 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 on 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 vapors of ether, gasoline and other odorous substances with an unpleasant odor).

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

There may also be pain in the bones when pressing or tapping (leukemia). Often, with blood diseases, the spleen is involved in the pathological process, then severe pain in the left hypochondrium, and if the liver is involved - in the right hypochondrium.

There may be enlarged and painful lymph nodes and tonsils.

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

Upon examination, the patient's condition is determined. Extremely severe cases can occur in the final stages of many blood diseases: progressive anemia, leukemia. Also, upon examination, pallor of the skin and visible mucous membranes is revealed, with iron deficiency anemia the skin has an “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 it is cherry red. With hemorrhagic diathesis, hemorrhages are visible on the skin and mucous membranes. The state of trophism of the skin also changes. With iron deficiency anemia, the skin becomes dry, flaky, hair becomes brittle and split.

When examining the oral cavity, atrophy of the tongue papillae 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 necrotizing tonsillitis and stomatitis (acute leukemia).

Palpation reveals tenderness of flat bones (leukemia), enlarged and painful lymph nodes (leukemia), an enlarged spleen (hemolytic anemia, acute and chronic leukemia). Percussion can also reveal an enlarged spleen, and auscultation reveals a friction sound of the peritoneum above the spleen.

Laboratory and instrumental research methods.

Morphological blood test: general blood analysis(determining the number of red blood cells and the hemoglobin content in them, determining the total number of leukocytes and the ratio of individual forms among them, determining the number of platelets, erythrocyte sedimentation rate). With iron deficiency anemia, the level of hemoglobin and the number of red blood cells decrease unevenly, and 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. Changes in leukocytes (qualitative and quantitative composition) are observed in leukemia.

Morphological assessment of red blood cells allows us to identify anemia.

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

More accurate information about the composition of the bone marrow gives trepanobiopsy, when the column of the ilium is cut out along 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 allows for a more accurate assessment of it.

Enlarged lymph nodes are often punctured, and 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 system: lymphocytic leukemia, lymphogranulomatosis, lymphosarcomatosis, detect tumor metastases and others. More accurate information can be obtained by biopsy of the lymph node or 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 departments hematopoietic system, identify the presence of extramarrow hematopoiesis in some bone marrow lesions.

Hemolysis assessment necessary when identifying hemolytic in nature anemia (determine free bilirubin, changes in the osmotic stability of red blood cells, the appearance of reticulocytosis).

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

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

Prevention of blood diseases

Prevention of diseases of the blood system consists of the following: timely diagnosis and treatment of diseases that are accompanied by blood loss (hemorrhoids, peptic ulcer, erosive gastritis, nonspecific ulcerative colitis, uterine fibromatosis, hernia hiatus diaphragm, intestinal tumors), helminthic infestations, viral infection, if they cannot be cured, then it is recommended to take iron supplements, vitamins (especially B12 and folic acid) and, accordingly, use foods containing them in food; 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 monitoring blood tests.

To prevent diseases of the blood coagulation system, family planning is used (prevention of hemophilia), prevention of hypothermia and stressful situations; vaccinations and blood tests are contraindicated. bacterial antigen, alcohol (for hemorrhagic vasculitis), refusal to carry out unnecessary blood transfusions, especially from different donors.

To prevent leukemia, it is necessary to reduce, if any, exposure to harmful factors, such as ionizing and non-ionizing radiation, varnishes, paints, benzene. For prevention severe conditions and complications, you do not need to self-medicate, but consult a doctor if any symptoms appear. If possible, try to undergo annual medical examination, 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 anemias
Anemia due to enzyme disorders
Aplastic and other anemias
Bleeding disorders, purpura and other hemorrhagic conditions
Other diseases of the blood and hematopoietic organs
Selected disorders involving the immune mechanism

Anemia is a polyetiological disease characterized by changes external signs(pallor of the skin, mucous membranes, sclera, often masked by jaundice), the appearance of disorders muscular system(weakness, decreased tissue turgor), abnormalities in the central nervous system (lethargy, apathy, mild irritability), functional disorders of cardio-vascular system(tachycardia, expansion of boundaries, the appearance of systolic murmur in Botkin’s point and the apex of the heart), the development of hepato- and splenomegaly, changes in the morphology of red blood cells (decrease in volume, change in shape, osmotic resistance), changes in the content of other cellular forms (leukocytes, platelets) of bone punctate brain, electrolyte metabolism and iron and magnesium content in blood serum.

The classification is as follows.

1. Deficiency anemia: iron deficiency, vitamin deficiency, protein deficiency.

2. Hypo- and aplastic anemia: congenital Fanko-ni anemia, Dabionda-Biekfen anemia, acquired anemia.

3. Hemolytic anemia: spherocytic, sickle cell, autoimmune.

By severity:

1) mild anemia: hemoglobin within 90 – 110 g/l, the number of red blood cells decreases to 3 minutes;

2) moderate anemia: hemoglobin 70–80 g/l, red blood cells up to 2.5 min;

3) severe anemia: hemoglobin below 70 g/l, red blood cells below 2.5 min. According to the functional state of erythropoiesis:

a) regenerative anemia: retiulocytes more than 50%;

b) hypo- and aregenerative anemias: low retilunocytosis, inadequate severity of anemia. According to the course: acute phase, subacute and chronic course.

Iron-deficiency anemia

Iron deficiency anemia is a disease that is caused by iron deficiency in the blood serum, bone marrow and depot, which leads to the development of trophic disorders in tissues. The development of anemia is preceded by latent tissue iron deficiency. It is more common in women than in men, in 14% of women of childbearing age living in the middle zone.

Etiology: The causes of iron deficiency anemia are chronic blood loss, insufficient initial iron levels, which manifest themselves during puberty; impaired absorption and intake of iron from food. Most often, several unfavorable factors are combined. Agistral and enthologenic anemias are often accompanied by a deficiency of not only iron, but also vitamin B12, folic acid, and proteins.

Classification:

1) chronic posthemorrhagic;

2) conditioned hemoglobinuria and hemosiderinemia;

3) iron deficiency in donors (withdrawal of 400–500 ml of blood is accompanied by a loss of 200–250 mg of iron.).

Clinic. History of insufficient, incorrect, one-sided nutrition, frequent illnesses. Dryness, roughness of the skin, brittle hair, pallor of the mucous membranes, atrophy of the papillae of the tongue; functional changes gastrointestinal tract, leading to spasms of the esophagus, accelerated intestinal motility, spleno- and hepatomegaly.

Changes in the morphology of erythrocytes and biochemical parameters of the blood serum, anisocytosis, poikilocytosis, a decrease in the osmotic ability of erythrocytes, a decrease in the concentration of serum iron, an increase in the copper content in the blood serum.

In the development mechanism clinical manifestations In iron deficiency anemia, tissue hypoxia and decreased activity of most enzymes are of paramount importance.

Patients experience severe weakness, darkening before the eyes when changing body position, headaches, dizziness, fainting, shortness of breath, palpitations during minor physical exertion, increased tooth decay, and smoothness of the papillae of the tongue. In severe cases - disturbance, discomfort when swallowing (Bechterew's symptoms) of dry and solid food, crimson coloration of the tongue, atrophic changes in the pharynx and esophagus, spastic narrowing of the upper part of the esophagus, fragility, formation of longitudinal or transverse striations along the nail plate, koilonychia. A distortion of the sense of taste (addiction to honey, tooth powder, chalk, dry cereal, coal, lime, ice, the smell of gasoline, kerosene) indicates a violation of peripheral taste sensitivity. Patients may complain of muscle weakness, urinary urgency, and enuresis. Hyporegeneration of blood cells is caused by a decrease in the proliferative capacity of the bone marrow and ineffective hematopoiesis. Differential diagnosis includes thalassemia, posthemorrhagic and infectious anemia.

Treatment

The principles of treatment are as follows.

1. Active mode.

2. Balanced diet.

3. Iron supplements in combination with ascorbic acid and copper.

4. Aerotherapy, massage, gymnastics.

5. Food enzymes.

6. Blood transfusion when the hemoglobin content is less than 60 g/l, iron supplements are prescribed orally between meals, since during this period the better absorption. In case of iron intolerance (decreased appetite, nausea, vomiting, epigastric pain, dyspepsia, allergic dermatoses), iron preparations are administered parenterally to avoid damage to the gastrointestinal mucosa. Iron preparations include hemostimulin, ferroplex, sorbifer durulez, ferrum lek. Prevention of anemia should be carried out for donors, women with heavy menstruation, frequent pregnancies, girls during puberty, with frequently recurring bleeding.

Vitamin deficiency anemia

Pernicious anemia (Addison-Birmer disease) is caused by a deficiency of vitamin B12, manifested by damage to the hematopoietic, digestive and nervous systems. Occurs more often in old age, with equal frequency in men and women.

Etiology. Vitamin deficiency is rarely exogenous, more often endogenous, associated with their increased consumption (helminthiases) and impaired absorption of various natures (stomach disease, malabsorption syndrome). Malabsorption of vitamin B 12 is most often caused by atrophy of the gastric mucosa and the absence or decrease in the secretion of internal factor, hydrochloric acid, and pepsin. There is a hereditary predisposition associated with impaired secretion of the internal factor; violation of immune mechanisms (detection of antibodies to own cells). Pernicious anemia can occur after gastrectomy or resection.

Clinic

In patients with pernicious anemia, the skin acquires a lemon-yellow tint, and spotted-brown pigmentation may form. Patients complain of weight loss caused by anorexia, and possibly an increase in body temperature. In half of the patients, symptoms of glossitis appear, sometimes the mucous membranes of the cheeks, gums, pharynx, esophagus are affected, diarrhea develops, the liver is enlarged, the spleen is often enlarged - hepatosplenomegaly; shortness of breath, palpitations, extrasystole, weakness, dizziness, tinnitus. Characteristic for pernicious anemia rheumatic syndrome, which is caused by damage white matter spinal cord. In severe cases, signs of damage to the posterior columns of the spinal cord appear, an uncertain gait, impaired coordination of movements, ataxia, hyperreflexia, and tone of the feet. Rare but dangerous symptoms are mental disorders, maximum outbursts, and paranoid states.

Differential diagnosis carried out with familial megaloblastic anemia.

Survey plan.

2. Examination of feces for occult blood.

3. Blood test for reticulocytes, platelets.

4. Determination of iron content in the blood (if iron deficiency anemia is suspected).

5. Fibrogastroduodenoscopy.

6. Colonoscopy (if irrigoscopy is not possible).

7. X-ray examination of the lungs.

8. Sternal puncture, myelogram study.

9. Biochemical research blood ( total protein and protein fractions, bilirubin, transaminases).

10. Study of the secretory function of the stomach.

Principles of treatment.

1. Diet therapy.

2. Purpose of B 12 and folic acid.

3. Enzyme therapy.

4. Anabolic hormones and insulin.

5. Stimulating therapy.

The best results are obtained by treatment with vitamin B 12 (cyanocobolamine). In severe cases, intravenous administration of the drug at 100–200 mcg for a week. The course dose is 1500–3000 mcg. In severe cases and in the presence of antibodies, corticosteroids are indicated.

Hypo- and aplastic anemia

A group of diseases of the blood system, the basis of which is a decrease in the production of bone marrow cells, most often of three cell lines: erythrocyte-, leuko- and thrombocytopoiesis.

Diagnostic criteria. Congenital Fanconi anemia: in the analysis there is a gradual increase in pallor of the skin and mucous membranes, weakness, lethargy, combined with a lag in physical development, strabismus, hyperreflexia.

IN early childhood melanin brown pigmentation of the skin appears, anomalies of the bones, forearms are detected, thumbs hands, spinal deformity, dwarfism, combined with malformations of the heart, kidneys (heart, renal failure), sensory organs, central nervous system (microcephaly); changes in the blood: drop in hemoglobin, pancytopenia, decrease in reticulocytes, deficiency of glucose-6-phosphate dehydrogenase, decreased activity of alkaline phosphatase, polysaccharides.

Congenital Estren–Dameshek anemia. History: manifests itself at an early stage of development of children with gradual development pallor, irritability, apathy; peculiar appearance: blond hair, snub nose, wide-set eyes, thickening upper lip with a bright red border; slowing down the rate of ossification in the wrists; spleno- and hepatomegaly, changes in the blood are expressed; increasing iron deficiency anemia; in bone marrow puncture: pallor of the brain due to the development of hypoplastic erythropoiesis.

Acquired hypo- and aplastic anemia. History: develops after viral infections, with a predisposition to allergic reactions to medications and toxins. Characterized by rapid development of clinical symptoms; temperature reaction (low-grade fever), pallor, skin rash, exanthema on the mucous membranes, stomatitis and sore throat, bloody stools, weakness, anorexia, shortness of breath, possible cerebral hemorrhages, adrenal glands:

1) changes in the blood: pancytopenia, a sharp decrease in hemoglobin, hyperchromia and macrocytosis of erythrocytes, iron content in the blood serum is normal or increased;

2) in bone marrow aspirate: fatty degeneration, poverty of formed elements, absence of young forms of megalocaryocytes.

Survey plan.

1. General analysis blood, urine.

3. Sterile puncture with myelography examination.

4. Fibrogastroduodenoscopy, colonoscopy, ultrasound examination of the liver, pancreas, kidneys (to exclude neoplasms).

Differential diagnosis. Inhibition of hematopoiesis can occur with osteosclerosis and osteomyelofibrosis. It is also necessary to differentiate aplastic (hypoplastic) anemia from acute leukemia and Werlhof's disease.

Principles of treatment.

1. Transfusion of red blood cells in acute forms.

2. Plasmophoresis with injection fresh frozen plasma, albumin or rheopolyglucin.

3. Impact on the vascular wall (dicinone, serotonin, rutin, ascorbic acid– askorutin).

4. Glucocorticoids along with massive doses of antibiotics; B vitamins, folic acid.

5. Aminocaproic acid, anabolic hormones (reta-bolin).

6. Splenectomy.

Hemolytic anemia

These are anemias that develop due to the destruction of red blood cells.

Etiology– a group of acquired and hereditary diseases characterized by intracellular or intravascular destruction of red blood cells. Autoimmune hemolytic anemia is associated with the formation of antibodies to the red blood cells' own antibodies.

Diagnostic criteria: hereditary microspherocytic anemia (Minkowski-Shoffir disease):

1) in the anamnesis: the first symptoms are detected at any age, they begin as a result of an existing congenital defect in the lipoid structures of the erythrocyte membrane, therefore it is important to identify relatives suffering from anemia;

2) pallor with a lemon-yellow tint, congenital stegein (tower skull, wide bridge of the nose, high palate, lethargy, weakness, decreased appetite up to anorexia, dizziness);

3) changes in the cardiovascular system, palpitations, shortness of breath, systolic murmur;

4) changes in the gastrointestinal tract: abdominal pain, colic and significant enlargement and hardening of the liver and spleen;

5) ulcers on the legs - changes in the blood: a decrease in the number of red blood cells to 2.5 minutes, hemoglobin to 70 g/l, an increase in retinulocytes to 30–50%, a decrease in the minimum osmotic resistance of red blood cells with an increased maximum, an increase in the level of indirect bilirubin, urobillinogen urine, stercobillin in the skin;

6) in bone marrow punctate – inhibition of the erythroid germ.

Sickle cell anemia

Anemia develops in early age. Family history and detection of abnormal hemoglobin in relatives are important:

1) pallor or yellowness of the skin, mucous membranes, sclera, asthenia of the body; characteristic appearance: short body, long thin limbs, narrow shoulders and hips, tower skull, big belly, ulcers on the extremities, hepatosplenomegaly, expansion of the borders of the heart, arrhythmia, systolic murmur;

2) changes in the blood: normochromic anemia 2.5–3 min, hemoglobin S or its combination with hemoglobin F, anisocytosis, poikilocytosis, target-like erythrocytes, deficiency of the enzyme glucose-6-phosphate dehydrogenase.

Autoimmune anemia:

1) history: anemia develops acutely or gradually after viral, bacterial infections, against the background of rheumatism, cirrhosis of the liver, lymphagranulomatosis, etc.;

2) pallor of the skin and mucous membranes, jaundice in 75% of cases, elevated body temperature, weakness, drowsiness, excitability, headaches, abdominal pain, back pain, splenomegaly, hematuria;

3) changes in the blood: decreased levels of hemoglobin, red blood cells, reticulocytes, bilirubinemia, increased serum iron concentration; positive reaction Coombs (detection of antibodies to red blood cells);

4) in urine: hemoglobinuria;

5) in bone marrow punctate: irritation of the erythrocyte process.

Survey plan.

1. General analysis of blood, urine, feces.

2. Blood test for reticulocytes, platelets.

3. Urine analysis for urobilin and bilirubin.

4. Biochemical analysis for urea, creatinine, transaminases (AIT, AST), bilirubin, total protein and protein fractions.

5. Study of osmotic resistance of erythrocytes.

6. Fibrogastroduodenoscopy.

7. Ultrasound examination of the liver, pancreas and gall bladder.

8. Coolibs reaction (if autoimmune anemia is suspected).

Differential diagnosis. Conducted from acute leukemia, Werlhof's disease, sepsis, and other hemolytic anemias in which spherocytosis is not detected and the osmotic resistance of erythrocytes increases (thalassemia, etc.).

Principles of treatment.

2. Transfusion of red blood cells, ear blood.

3. Corticosteroids.

4. Glucose 5% with insulin and vitamins B, B2, B12, B6, C.

5. If there is no effect - immunosuppressants, splenectomy, laser irradiation.

Hemorrhagic diathesis is a group of diseases characterized by impaired hemostasis (vascular, platelet or plasma) and manifested by an increased tendency to bleeding and hemorrhage.

Etiology

Heredity hemorrhagic conditions determined by abnormalities of megakaryocytes and platelets, defects in plasma coagulation factors, and inferiority of cervical blood vessels.

Acquired hemorrhagic diathesis is caused by disseminated intravascular coagulation syndrome, toxic-infectious conditions, liver diseases, and the effects of medications.

Classification

Diets differentiate.

I. A disease caused by impaired vascular hemostasis (vasopathy).

1. Shenain-Henoch disease (simple, rheumatoid, abdurate and fulminant purpura):

1) simple form;

2) chronic form.

2. Hereditary familial simple purpura (Davis).

3. Anular telangiectatic purpura of Mabocchi.

4. Necrotizing Sheldon's purpura.

5. Hyperglobulinemic Waldenström's purpura.

6. Hereditary hemorrhagic telangiectasia.

7. Louis-Barr syndrome (capillary telangiectasia of the conjunctiva with ataxia and chronic pneumonia).

8. Kasabach-Merritt syndrome.

9. Scurvy and Mimer-Barny disease.

II. Diseases caused by a violation of the platelet mechanism of hemostasis (thrombocytopathy, thrombocytopenia):

1) hemorrhagic thrombocytopathy, Werlhof's disease:

a) acute form;

b) chronic form (continuous and recurrent);

2) amegakaryocytic thrombocytopenic purpura (Landolt);

3) autoimmune thrombocytopenia of various origins;

4) thrombocytopenic hymphragic purpura with acquired autoimmune hemolytic anemia(Feemer-Evans syndrome);

5) thrombocytopenic purpura with a chronic purulent shade and exudative diathesis (Ondrich syndrome);

6) thrombotic thrombocytopenic purpura of Moshkovich;

7) thrombocytopenia in geangiomas (Kasabach-Merritt syndrome);

8) hereditary properties of thrombopathy:

a) Glanuman thrombasthenia;

b) von Willibrand thrombopathy;

9) thrombocytopathy in combination with a violation of coagulation factors.

III. Diseases caused by disorders of blood clotting factors (coagulopathy):

1) hemophilia A (factor VIII deficiency):

a) hereditary;

b) family;

c) sporadic;

2) hemophilia B (factor IX deficiency):

a) hereditary;

b) family;

c) sporadic;

3) hemophilia C (factor XI deficiency);

4) pseudohemophilia due to hypoprothrombinemia:

a) idiopathic hypoprothrombinemia;

b) secondary hypoprothrombinemia (hemorrhagic disease of newborns, impaired absorption of vitamin K, liver disease, poisoning with chloroform, phosphorus, arsenic);

5) Ouren's pseudohemophelia:

a) congenital form;

b) acquired form;

6) pseudohemophelia due to deficiency of factor VII:

a) congenital form;

b) acquired form;

7) pseudohemophelia due to a lack of fibrinogen (afibrinogenemia):

a) congenital form;

b) acquired form (DIC syndrome);

8) pseudohemophelia due to lack of factor X;

9) pseudohemophelia due to lack of fabrinase;

10) pseudohemophelia due to excess anticoagulants:

a) idiopathic;

b) immunoallergic;

c) acquired forms.

Hemorrhagic vasculitis(Shenain-Henoch disease, capillary toxicosis, anaphylactic purpura) is an infectious-toxin-allergic disease based on generalized hyperegic inflammation of blood vessels.

Etiology

The cause of the acute inflammatory process of small joints of the skin, joints of the digestive tract and kidneys is not fully understood.

Degree of activity – I, II, III.

Course: acute, subacute, chronic, recurrent.

Outcome: recovery, transition to a chronic form, outcome to chronic nephritis (A. S. Kalinichenko, 1970).

Diagnostic criteria

Clinical:

1) hemorrhagic skin syndrome: the rash is usually located symmetrically, characterized by stages of the rash, localized on the extensor surfaces of the limbs, around the ankles and knee joints, in the area of ​​​​the feet, less often the hips; rashes are usually polymorphic: hemorrhagic papules, erythrimatous papules, spots; at the beginning of the disease, the rashes are urtic in nature, later they become hemorrhagic, up to necrosis, and relapses are typical;

2) articular syndrome: joint lesions have a migratory polyatric nature with a predominant localization in the legs, ankles, elbows, wrist joints, and joint damage is rarely symmetrical;

3) abdominal syndrome: cramping abdominal pain of varying intensity; pain may be accompanied by intestinal and kidney bleeding.

Laboratory research: hematological changes: leukocytosis, neutrophilia, eosinophilia, accelerated ESR, platelet count is sometimes slightly reduced; blood clot retraction, bleeding duration and blood clotting time are not impaired; hypercoagulation is often detected; urine test: in acute period diseases often reveal morning proteinuria, hematuria; There may be an admixture of blood in the stool with abdominal syndrome.

Examination plan:

1) general analysis of blood, urine, feces;

2) study of platelet clotting time;

3) determination of coagulogram;

4) examination of feces for occult blood (Gregersen reaction).

Differential diagnosis

It is carried out with thrombocytopathies, thrombocytopenias, coagulopathy, toxic drug-induced vasculitis, allergic and infectious diseases.

Principles of treatment

1. Hospitalization and bed rest for at least three weeks.

2. Diet excluding cocoa, coffee, citrus fruits, strawberries, etc.

3. Heparin therapy.

4. Nicotinic acid in combination with heparin.

5. Prednisolone.

6. Plasmapheresis (for chronic vasculitis).

Thrombocytopathies are a quantitative and qualitative deficiency of the platelet component of hemostasis, clinically manifested by hemorrhagic syndromes.

Etiology

Depending on the genesis, two groups are distinguished:

1) thrombocytopenia – a decrease in the number of platelets (Werlhoff, Wilbrand-Jurgens, Frank, Kasabach-Merritt disease);

2) thrombocytopathies - a violation of the properties of platelets. In the vast majority of cases, thrombocytopenia is observed, which is based on an immunoallergic conflict.

Classification

By type: primary (idiopathic) and secondary (symptomatic) thrombocytopenia.

Nosological forms: isoimmune, transimmune, heteroimmune, autoimmune.

Werlhof's disease

Classification of idiopathic thrombocytopenic purpura (Werlhof's disease)

Course: acute (up to 6 months); chronic: with rare relapses, with partial relapses, continuously relapsing.

Clinical picture of purpura: dry purpura (skin syndrome); wet purpura (skin syndrome and bleeding).

Immunological tests: positive, negative.

Period: exacerbation, clinical remission, clinical-gamotological remission.

Complications: uterine, gastric, intestinal bleeding, posthemorrhagic encephalopathy, etc.

Diagnostic criteria

Clinical: pallor of the skin and immune membranes:

1) hyperplastic syndrome: enlarged spleen, less often – liver;

2) hemorrhagic syndrome: hemorrhages in the skin, mucous membranes (asymmetrically located, of different shapes and sizes from petechiae to ectomosis, bleeding from different organs(nasal, uterine, intestinal, etc.), positive endothemial tests (symptoms of tourniquet, pinch).

Laboratory criteria:

1) in a general blood test, a decrease in the number of platelets, changes in the morphology of the plates and their functional properties (adhesion, aggregation); impaired blood clot retraction; increased duration of bleeding, slower blood clotting; decrease in the number of red blood cells and raticunocytes during bleeding;

2) change in the myelogram: hyperplasia of the megakaryocyte lineage with impaired functional activity of megaparyocytes;

3) immunological: the presence of antiplatelet antibodies. Survey plan.

1. General analysis of blood, urine, feces.

2. Blood clotting time, bleeding duration, platelet count.

3. Coagulogram.

4. Feces for occult blood (Gregersen reaction).

5. Examination of bone marrow puncture (myelogram).

6. Immunological studies for the presence of antiplatelet antibodies.

Differential diagnosis

It is necessary to distinguish idiopathic thrombocytopenic purpura from allergic anemias, primary diseases liver in combination with splenomegaly, systemic lupus erythematosus, genetically determined thrombocytopenia.

Treatment

Principles of treatment of thrombocytopenia:

1) prevention of bruises and injuries;

2) antibiotics for bacterial infections;

3) transfusion of plasma and large doses of β-globulin;

4) corticosteroids;

5) splenectomy;

6) immunosuppressants (azothiopril, vancristine). Principles of treatment of thrombocytopathies:

1) E-aminocaproic acid, synthetic contraceptives, (bisecurin, microfollin), magnesium sulfate 25% intramuscularly, magnesium thiosulfate orally;

2) locally, subcutaneously or intramuscularly, adrenochrome monosemicarbazone (adroxon, chromadrone, adrenoxyl), dicyone;

3) intravenous administration of platelet mass.

Coagulopathies are disorders of hemostasis, which are based on a deficiency of certain plasma coagulation factors.

Etiology

Hereditary coagulopathies (they are correctly called hemophilia) are caused by a genetically determined decrease or change in plasma components of hemostasis. Acquired coagulopathies occur in infectious diseases, liver and kidney diseases, severe enteropathies, rheumatoid arthritis and etc.

Classification

Classifications of hereditary coagulopathies.

1. Hemophilia: A-deficiency of factor VIII (synthyhemophilic globulin); B-deficiency of factor IX (Christmas); C-deficiency of factor XI (precursor of plasma thromboplastin); D-deficiency XII (Hagemani).

2. Parahemophilia: deficiency of factor V (proaccelerin); deficiency of factor VII (proconvertin); deficiency of factor II (pro-trolobin); factor X deficiency (Stewart-Prower).

3. Impaired fibrin formation, deficiency of factor I (fibrinogen). Forms of flow: light, heavy, hidden.

Diagnostics

Clinical diagnostic criteria are pallor of the skin and immune membranes; hemorrhagic syndrome: hamarthrosis, hemorrhage into soft tissues due to trauma to the skin and mucous membranes (extensive hematomas); hematuria; internal hemorrhages.

Laboratory diagnostic criteria - hematological: anemic syndrome (decrease in the number of red blood cells and hemoglobin, hypochromia, reticulocytosis with bleeding), hypocoagulation syndrome of blood clotting disorders (according to Lee-White more than 10 minutes), increased recalcification time (more than 250 s), increased plasma tolerance to heparin (more than 180 s), decrease in plasma factors.

Survey plan.

1. General analysis of blood and urine.

2. Determination of blood clotting time and platelet count.

3. Coagulogram, determination of antihemophilic globulin (AGG).

4. X-ray of the affected joints.

Differential diagnosis

Carry out with thrombocytopathies, hemorrhagic vasculitis, hemarthrosis with rheumatism.

Treatment

The principles of treatment are as follows:

1) increase in the blood level of AGG: administration of AGG concentrates, fresh frozen plasma, concentrates containing factor IX; desmopressin (increasing the level of factor VIII);

2) transfusions, venipuncture against the background of the administration of epsilon-aminocaproic acid;

3) prevention of injuries and use of drugs containing aspirin.

1. The most common is anemic syndrome(anemia, anemia), which is clinically manifested mainly by pallor of the skin and mucous membranes, as well as signs of insufficient oxygen supply to organs and tissues, such as: palpitations and shortness of breath (especially during physical effort), tinnitus, dizziness, weakness, increased fatigue , fainting, etc.. From a hematological point of view, anemia can be divided into hypo- and hyperchromic and into aregenerative and regenerative.

In hypochromic anemia, the decrease in the amount of hemoglobin is more pronounced than the decrease in the number of red blood cells, and therefore the color index in these cases is less than one (for example, essential hypochromic anemia, anemia after blood loss, chlorosis). In hyperchromic anemia, the amount of hemoglobin is reduced less than the number of red blood cells, and therefore the color index is greater than one (pernicious anemia, hemolytic anemia).

In aregenerative or aplastic anemia, there are no signs of red blood regeneration in the peripheral blood (and in advanced cases, in the bone marrow), i.e., there are no reticulocytes, polychromatophils, macrocytes, erythroblasts, etc. Since the entire bone marrow suffers, then leukopenia (granulocytopenia) and thrombocytopenia are simultaneously observed, and a picture of so-called panmyelophthisis may develop. Aplastic anemia is not an independent form, but only a variant of anemia of any origin.

With regenerative anemia, there are always more or less pronounced signs of regeneration. This group includes primarily pernicious anemia, as well as hemolytic anemia.

A typical example of the hyperchromic regenerative nature of anemia is the so-called pernicious anemia. Clinically, it is characterized by pronounced pallor of patients in the absence of a decline in nutrition, the presence of gastric achylia and changes in the nervous system (spinal cord). The main hematological features of pernicious anemia are the following: a very sharp decrease in the number of red blood cells (often less than 10% of the normal number remains), the predominance of large cells among them (macro- and megalocytes), the presence of megaloblasts, a high color index, leukopenia, thrombopenia.

2. The opposite of anemic is polycythaemic syndrome. It is based on increased erythropoiesis. Clinically, it is expressed mainly by disturbances in the functioning of the circulatory system: a sharp increase in the color of the skin of the face and hands and especially the mucous membrane of the lips and oral cavity and pharynx to a dark cherry-red color, congestion in the internal organs, headaches, dizziness, hemorrhages, thrombosis. From a hematological point of view, this syndrome is characterized by a sharp increase in the number of red blood cells in 1 mm3 of blood (up to 12 - 14 million), clear signs of regeneration (reticulocytes, erythroblasts), often neutrophilic leukocytosis (increased leukopoiesis), and increased blood viscosity.

Polycythemia often develops as compensatory process due to oxygen starvation of the body. In these cases, we can talk about erythrocytosis as a phenomenon similar to leukocytosis. But polycythemia is also observed as an independent pathological process without any accompanying diseases that can explain it. Such cases, by analogy with leukemia, are called erythremia.

3. Leukemic syndrome- leukemia (bleeding) or leukemia - develops due to hyperplastic proliferation of leukoblastic tissues of the hematopoietic system. Clinically, it is manifested mainly by enlargement of the lymph nodes and spleen, and hematologically it is characterized by an increase in the number of white blood cells and the appearance among them of young forms or forms unusual for peripheral blood.

According to the three types of leukoblastic tissue, usually affected in isolation, three forms of leukemia or leukemia are observed: myeloid leukemia or myelosis, lymphatic leukemia or lymphadenosis and reticuloendothelial (monocytic) leukemia or reticuloendotheliosis.

Sometimes the leukemic process develops and proceeds quickly, like an acute septic disease, and changes in the lymph nodes and spleen do not have time to be noticeably revealed, acute leukemia. Much more often the process takes a long time and in such cases gives a typical picture of chronic leukemia. Finally, there are cases of hyperplasia of one or another leukoblastic tissue without corresponding changes in the white blood, so-called pseudoleukemia or aleukemic leukemia (myelosis, lymphadenosis and reticuloendotheliosis).

The most common chronic leukemic myelosis is characterized clinically by a sharp enlargement of the spleen, as well as the liver (due to the growth of metaplasia of myeloid tissue in it) in the absence or slight enlargement of lymph nodes, and a hematologically huge (up to several hundred thousand in 1 mm3) increase in the amount of white blood cells of the myeloid series with the appearance among them of a large number (up to 50%) of immature forms at different stages of development.

Chronic leukemic lymphadenosis is manifested clinically by pronounced multiple enlargement of lymph nodes and, to a lesser extent, enlargement of the spleen and liver (growth in it lymphatic tissue) and hematologically a huge increase (hundreds of thousands to a million or more per 1 mm3) in the number of white blood cells due to small lymphocytes (up to 90 - 99% of the total).

Chronic leukemic reticuloendotheliosis is very rare disease- clinically it also gives an enlargement of the lymph nodes, spleen and liver (due to hyperplasia of the reticuloendothelial tissue in them); hematologically, it is characterized by a significant increase in the number of leukocytes (up to several tens of thousands per 1 mm3) with the appearance in the blood of a large number of large cells such as monocytes.

4. Hemorrhagic syndrome characterized by a pronounced tendency to bleeding and hemorrhage, occurring spontaneously or caused by minimal trauma. Three main mechanisms underlie hemorrhagic phenomena:

A) Toxicosis of capillary vessels, caused by certain factors (infection, allergy, vitamin deficiency) and leading to increased fragility and patency of the capillary walls; the composition and properties of the blood itself remain without deviations from the norm. These include the so-called purpura purpura simplex (skin hemorrhages only), purpura rheumatica (accompanied by joint lesions), purpura abdominalis (hemorrhage into the internal organs abdominal cavity), purpura fulminans (with a very fast lightning current), as well as scurvy, etc.

B) Thrombocytopenia, resulting in prolongation of bleeding time and lack of compression (retraction) of the blood clot; Blood clotting in these cases does not change. This includes Werlhof's disease (morbus Werlhofii) or Frank's essential thrombopenia.

C) Changes in blood chemistry, namely a slowdown in blood clotting due to disruption (slowdown) of the process of formation of thrombin (fibrin enzyme) due to a lack of thrombokinase; the rest of the blood is normal, in particular the platelet count, clot retractility, and duration of bleeding are normal. Belongs here