home All hormones Semiotics and syndromes of damage to the blood system in children. Oncogenetic syndromes of the hematopoietic and immune systems

Semiotics and syndromes of damage to the blood system in children. Oncogenetic syndromes of the hematopoietic and immune systems

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 “acute” posthemorrhagic anemia"", "chronic posthemorrhagic anemia", "megaloblastic anemia", "acute leukemia", "chronic myeloid leukemia", "chronic lymphocytic leukemia", ideas about 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. draw up a plan for additional, most informative studies 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 for the appearance of most clinical signs is 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, fatigue and decreased performance;

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

- shortness of breath when physical activity, predominantly inspiratory;

– heartbeat;

– stabbing pains in the region of the heart;

– fainting conditions;

– loss of appetite;

– flashing “flies” before the eyes;

– persistent headaches; memory loss, drowsiness.

1.2. Physical research

External research:

Paleness of the 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 the leukocyte formula to myelocytes and/or blasts:

Hepatosplenomegaly;

Enlarged lymph nodes, rare;

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 due to hemorrhagic vasculitis, periarteritis nodosa, infectious diseases, and exposure to 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 to blood vessels;

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

Diseases of the gastrointestinal tract (peptic ulcer 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

The following syndromes are characteristic of posthemorrhagic anemia:

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;

Superficial 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 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 still normal indicators hemoglobin and red blood cells. 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 of the esophagus, 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 disease);

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

Genitals (long and heavy menstruation, endometriosis, uterine fibroids, malignant tumors of the 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;

Resection small intestine;

Gastric resection.

4. Impaired intake of iron from food:

Vegetarianism;

Low socio-economic standard of living;

Frequent use 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 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;

Maybe moderate increase ESR.

2. Serum iron and ferritin are reduced.

3. Research bone marrow– 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: decrease in amplitude, negative teeth T in the chest 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).

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 vascular wall. Along with increased blood clotting and thrombus formation, bleeding from the gums and dilated veins of the esophagus is observed in polycythemia. 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). The erythrocyte sedimentation rate (ESR, a nonspecific laboratory test that reflects the ratio of different types of blood proteins) usually decreases. Ultrasound examination (ultrasound) internal organs evaluates the size of the liver and spleen, their structure for damage by tumor cells and the presence of hemorrhages.

Symptoms of diseases of the blood system are quite diverse and most of them are not specific (that is, they can also be observed in diseases of other organs and systems). It is precisely because the symptoms are nonspecific that many patients do not seek treatment. medical care in the first stages of the disease, and come only when there is little chance of recovery. However, patients should be more attentive to themselves and if they have doubts about their own health, it is better not to “delay” and wait until it “goes away on its own,” but to immediately consult a doctor.

So, let's look at the clinical manifestations of the main diseases of the blood system.

Anemia

Anemia can be an independent pathology or occur as a syndrome of some other diseases.

Anemias are a group of syndromes common feature which is a decrease in the level of hemoglobin in the blood. Sometimes anemia is an independent disease (hypo- or aplastic anemia, etc.), but more often it occurs as a syndrome in other diseases of the blood system or other body systems.

There are several types of anemia, the common clinical sign of which is anemic syndrome associated with oxygen starvation of tissues: hypoxia.

The main manifestations of anemic syndrome are as follows:

pallor of the skin and visible mucous membranes (oral cavity), nail bed;
increased fatigue, a feeling of general weakness and weakness;
dizziness, flashing spots before the eyes, headaches, tinnitus;
sleep disturbances, deterioration or complete absence appetite, sexual desire;
increased breathing, feeling of lack of air: shortness of breath;
palpitations, increased heart rate: tachycardia.

Manifestations iron deficiency anemia are caused not only by hypoxia of organs and tissues, but also by iron deficiency in the body, the symptoms of which are called sideropenic syndrome:

dry skin;
cracks, ulcerations in the corners of the mouth - angular stomatitis;
layering, brittleness, cross-striations of nails; they are flat, sometimes even concave;
burning sensation of the tongue;
perversion of taste, desire to eat toothpaste, chalk, ash;
addiction to some atypical odors: gasoline, acetone and others;
difficulty swallowing solid and dry foods;
in females - urinary incontinence when laughing, coughing; in children - ;
muscle weakness;
in severe cases - a feeling of heaviness, pain in the stomach.

B12 and folate deficiency anemia characterized by the following manifestations:

hypoxic or anemic syndrome (signs described above);
signs of damage to the gastrointestinal tract (aversion to meat food, loss of appetite, pain and tingling in the area of the tip of the tongue, taste disturbance, “varnished” tongue, nausea, vomiting, heartburn, belching, stool disorders - diarrhea);
signs of damage spinal cord, or funicular myelosis (headache, numbness in the limbs, tingling and crawling, unsteady gait);
psycho-neurological disorders (irritability, inability to perform simple mathematical functions).

Hypo- and aplastic anemia usually begin gradually, but sometimes they debut acutely and progress rapidly. The manifestations of these diseases can be grouped into three syndromes:

anemic (discussed above);
hemorrhagic (various sizes - dotted or in the form of spots - hemorrhages on the skin, gastrointestinal intestinal bleeding);
immunodeficiency, or infectious-toxic (persistent increase in body temperature, infectious diseases of any organs - otitis media, and so on).

Hemolytic anemia outwardly manifests itself as signs of hemolysis (destruction of red blood cells):

yellow coloration of the skin and sclera;
an increase in the size of the spleen (the patient notices a formation in the left side);
increased body temperature;
red, black, or brown urine;
anemic syndrome;
sideropenic syndrome.

Leukemia

In leukemia, cancer cells replace healthy cells in the bone marrow, the deficiency of which in the blood causes the corresponding clinical symptoms.

This is a group of malignant tumors that develop from hematopoietic cells. The altered cells multiply in the bone marrow and lymphoid tissue, oppressing and replacing healthy cells, and then enter the bloodstream and spread through the bloodstream throughout the body. Despite the fact that the classification of leukemia includes about 30 diseases, their clinical manifestations can be grouped into 3 leading clinical and laboratory syndromes:

tumor growth syndrome;
tumor intoxication syndrome;
hematopoietic suppression syndrome.

Tumor growth syndrome occurs due to the spread of malignant cells to other organs and systems of the body and the growth of tumors in them. Its manifestations are as follows:

swollen lymph nodes;
enlarged liver and spleen;
pain in bones and joints;
neurological symptoms (persistent severe headache, nausea, unrelieved vomiting, fainting, convulsions, strabismus, unsteadiness of gait, paresis, paralysis, and so on);
changes in the skin - formation of leukemia (tubercles) white, consisting of tumor cells);
inflammation of the gums

Tumor intoxication syndrome is associated with the release of biologically active substances that are toxic to the body from malignant cells, the circulation of cell decay products throughout the body, and changes in metabolism. Its signs are as follows:

malaise, general weakness, fatigue, irritability;
decreased appetite, poor sleep;
sweating;
increased body temperature;
itching of the skin;
weight loss;
joint pain;
renal edema.

Hematopoietic suppression syndrome occurs due to a lack of red blood cells in the bloodstream (anemic syndrome), platelets (hemorrhagic syndrome) or leukocytes (immunodeficiency syndrome).

Lymphomas

Malignant is a group of tumors of the lymphatic system that arise as a result of the formation of pathologically altered lymphoid cell, capable of uncontrolled proliferation (reproduction). Lymphomas are usually divided into 2 large groups:

Hodgkin's (Hodgkin's disease, or lymphogranulomatosis);
non-Hodgkin's lymphomas.

Lymphogranulomatosis– a tumor of the lymphatic system with primary damage to lymphoid tissue; makes up about 1% of all oncological diseases adults; Persons aged 20 to 30 and over 50 years of age are more often affected.

Clinical manifestations of Hodgkin's disease are:

asymmetrical enlargement of the cervical, supraclavicular or axillary lymph nodes (the first manifestation of the disease in 65% of cases); the nodes are painless, not fused to each other or to surrounding tissues, movable; as the disease progresses, the lymph nodes form conglomerates;
in every 5th patient, lymphogranulomatosis debuts with enlarged mediastinal lymph nodes, which is initially asymptomatic, then cough and chest pain, shortness of breath appear);
several months after the onset of the disease, symptoms of intoxication appear and steadily progress (fatigue, weakness, sweating, loss of appetite and sleep, weight loss, itchy skin, increased body temperature);
tendency to infections of viral and fungal etiology;
gradually all organs containing lymphoid tissue– pain occurs in the sternum and other bones, the liver and spleen increase in size;
in the later stages of the disease, signs of anemic, hemorrhagic syndromes and a syndrome of infectious complications appear.

Non-Hodgkin's lymphomas is a group of lymphoproliferative diseases with primary localization mainly in the lymph nodes.

Clinical manifestations:

usually the first manifestation is an enlargement of one or more lymph nodes; when palpated, these lymph nodes are not fused together and are painless;
sometimes, in parallel with the enlargement of the lymph nodes, symptoms of general intoxication of the body appear (weight loss, weakness, itching of the skin, increased body temperature);
a third of patients have lesions outside the lymph nodes: in the skin, oropharynx (tonsils, salivary glands), bones, gastrointestinal tract, lungs;
if the lymphoma is localized in the gastrointestinal tract, the patient is bothered by nausea, vomiting, heartburn, belching, abdominal pain, constipation, diarrhea, intestinal bleeding;
sometimes lymphoma affects central system, which is manifested by severe headaches, repeated vomiting that does not bring relief, convulsions, paresis and paralysis.

Myeloma

One of the first manifestations of myeloma is persistent bone pain.

Myeloma, or multiple myeloma, or plasmacytoma is a separate type of tumor of the blood system; originates from precursors of B lymphocytes that retain a certain ability to differentiate.

Main syndromes and clinical manifestations:

pain syndrome (pain in the bones (ossalgia), radicular pain between the ribs and in the lower back (neuralgia), pain in the peripheral nerves (neuropathy));
bone destruction syndrome (bone pain associated with osteoporosis, compression fractures bones);
hypercalcemia syndrome ( high content calcium in the blood - manifested by nausea and thirst);
hyperviscose, hypercoagulability syndrome(due to violation biochemical composition blood - headaches, bleeding, thrombosis, Raynaud's syndrome);
recurrent infections (due to immunodeficiency - recurring sore throats, otitis, pneumonia, pyelonephritis, and so on);
renal failure syndrome (swelling that first appears on the face and gradually spreads to the trunk and limbs, increased blood pressure, which cannot be corrected with conventional antihypertensive drugs, cloudiness of urine associated with the appearance of protein in it);
in the later stages of the disease - anemic and hemorrhagic syndromes.

Hemorrhagic diathesis

Hemorrhagic diathesis is a group of diseases whose common feature is increased bleeding. These diseases may be associated with disorders in the blood coagulation system, a decrease in the number and/or function of platelets, pathology of the vascular wall, and combined disorders.

Thrombocytopenia– decrease in platelet content in peripheral blood less than 140*10 9 /l. Main feature of this disease– hemorrhagic syndrome varying degrees severity, directly dependent on platelet levels. Usually the disease is chronic, but can also be acute. The patient pays attention to pinpoint rashes and subcutaneous hemorrhages on the skin that appear spontaneously or after injuries. Blood leaks through wounds, injection sites, and surgical sutures. Nosebleeds, bleeding from the digestive tract, hemoptysis, hematuria (blood in the urine) are less common; in women - heavy and long periods. Sometimes the spleen becomes enlarged.

Hemophilia- This hereditary disease, characterized by a blood clotting disorder due to a lack of one or another internal factor coagulability. Clinically

Anemic syndrome:

Anemic syndrome is a clinical and hematological condition caused by a decrease in hemoglobin content and a decrease in the number of red blood cells in the blood below their normal values. Depending on the degree of decrease in hemoglobin, mild (hemoglobin 90-110 g/l), moderate (hemoglobin 60-80 g/l), severe (hemoglobin below 60 g/l) forms of anemia are distinguished. IN clinical picture Anemia can be identified as symptoms that are inherent to one degree or another in all types of anemia, regardless of their origin. These symptoms are called general anemic syndrome, hemic hypoxia syndrome, or circulatory hypoxia. It is based on tissue hypoxia due to a decrease in the number of red blood cells and hemoglobin, and the reaction of the cardiovascular system to tissue hypoxia. Patients complain of weakness, tinnitus, gradually developing shortness of breath, palpitations, and sometimes pain in the heart area. Depending on the nature of the anemia, there may be more typical complaints. Patients with iron deficiency anemia may complain of taste distortion and addiction to unusual smells. They eat chalk, tooth powder, clay, ice, sand, dry cereals; They love the smell of acetone, varnish, paints, fuel oil, gasoline. Typical complaints for patients with B12-deficiency anemia are pain in the tongue, burning and tingling sensations in it; with prolonged anemia, signs of damage to the nervous system appear - discomfort in the lower extremities, muscle weakness, unsteadiness and uncertainty in gait. This is due to damage to the posterior and lateral columns of the spinal cord. Patients with hemolytic anemia may have complaints of transient jaundice; with intravascular hemolysis, there may be changes in the color of urine.

Hyperplastic syndrome

Manifestations of hyperplastic syndrome include enlargement of the lymph nodes in the mediastinum (in 8% of patients), which often occurs with compression syndrome: shortness of breath, swelling of the neck and chest, swelling and pulsation of blood vessels. Gingival hyperplasia (in 5% of patients) is usually observed in severe cases of the process and is regarded as an unfavorable prognostic sign. Sometimes it reveals ulcerative-necrotic changes in the oral cavity (in 8% of patients), which is explained by leukemic infiltration of the submucosal layer and malnutrition, tissue decay, the formation of ulcers and necrosis. Skin infiltrates in the form of reddish-bluish papular plaques located in the thickness of the dermis are also considered as manifestations of hyperplastic syndrome. Specific skin manifestations must be differentiated from nonspecific ones detected by pathological process: allergic, such as drug dermatitis and urticaria, and infectious-inflammatory - septic emboli, often observed in septicemia and representing inflammatory painful infiltrates with softening in the center, where a purulent-necrotic, sometimes hemorrhagic vesicle is formed. Skin changes often have a mixed inflammatory-specific nature and are observed in very severe cases of the process.

Leukemic hyperplasia and bone marrow infiltration lead to inhibition of normal hematopoiesis, resulting in anemia and thrombocytopenia. Severe anemia is observed in 20% of patients. Deep thrombocytopenia, detected in 35% of patients, and severe anemia in the initial period of the disease not only indicate rapid progression of the process with deep damage to normal hematopoiesis, but also to a certain extent indicate a delayed diagnosis.

Hemorrhagic syndrome, or a tendency to skin hemorrhage and bleeding of mucous membranes, occurs as a consequence of changes in one or more parts of hemostasis. This may be damage to the vascular wall, a violation of the structure, function and number of platelets, or a violation of coagulation hemostasis. When determining the causes of bleeding, it is necessary to take into account that some types of pathology are common, others are rare, and still others are extremely rare. From hereditary disorders hemostasis, the most common thrombocytopathies in therapeutic practice are hemophilia A, von Willebrand disease, hemophilia B, and vascular forms are telangiectasia. The most common causes of acquired forms of hemorrhagic syndrome are secondary thrombocytopenia and thrombocytopathies, DIC syndrome, deficiency of prothrombin complex factors and hemorrhagic vasculitis. Other forms are rare or very rare. It should be taken into account that in recent years, hemostasis disorders and, as a consequence, hemorrhagic syndrome are increasingly associated with taking medicines, disrupting platelet aggregation (antiplatelet agents) and blood clotting (anticoagulants), as well as psychogenic forms - neurotic bleeding and Munchausen syndrome.

Plethoric syndrome caused by an increased content of red blood cells, as well as leukocytes and platelets (plethora - plethora). This syndrome consists of: 1) subjective syndromes, 2) disorders of the cardiovascular system, 3) shifts in laboratory parameters.

1. Subjective symptoms of plethoric syndrome include: headaches, dizziness, blurred vision, angina pain, skin itching, erythromelalgia (sudden onset of hyperemia with a bluish tint of the skin of the fingers, accompanied by sharp pain and burning), sensations of numbness and chilliness of the limbs are possible.

2. Disorders of the cardiovascular system are manifested in changes in the color of the skin and visible mucous membranes, such as erythrocyanosis, and features of the color of the mucous membrane at the transition site soft palate into the solid (Cooperman's symptom), arterial hypertension, development of thrombosis, less often bleeding. In addition to thrombosis, swelling of the legs and erythromelalgia are possible. Circulatory disorders in the arterial system can lead to serious complications: acute myocardial infarction, strokes, visual impairment, and renal artery thrombosis.

3. Shifts in laboratory parameters are determined mainly by clinical analysis blood: there is an increase in the content of hemoglobin and red blood cells, an increase in hematocrit and blood viscosity, moderate leukocytosis with a shift leukocyte formula to the left, thrombocytosis, sharp slowdown in ESR.

Bone marrow hematopoiesis deficiency syndrome, or myelophthisis, can develop acutely when damaged by penetrating radiation, individual high sensitivity to antibiotics, sulfonamides, cytostatics, anti-inflammatory or analgesic drugs. Possible damage to all bone marrow hematopoiesis. Clinical manifestations: high fever, intoxication, hemorrhagic rash or bleeding, necrotic inflammation and ulcerative processes on the mucous membranes, local or generalized manifestations of infection or fungal diseases. In the peripheral blood, pancytopenia is observed in the absence of signs of blood regeneration, in the bone marrow puncture there is a depletion of cellular forms of all germs, a picture of cellular decay.

Syndrome secondary immunodeficiency characterized by recurrent infections that progress to chronic form, and occur with a slight but prolonged increase in body temperature.

Anemia can be an independent disease or a syndrome in other diseases of the blood system, chronic intoxication, infectious-inflammatory, immunopathological processes, acute and chronic blood loss. Anemia is characterized by a decrease in the number of red blood cells and hemoglobin in the peripheral blood.

In pediatric practice, the classification of anemic conditions by E.N. Mosyagina.

Classification of anemia

Anemia caused by a lack of hematopoietic factors:
Iron deficiency
Vitamin deficiencies
Protein deficient

Hypoplastic and aplastic anemia:

A) hereditary hypoplastic anemia:

1. With general damage to hematopoiesis (Fanconi anemia, Estren-Dameshek anemia).

2. With selective damage to erythropoiesis (Blackfan-Diamond anemia).

B) Acquired hypoplastic and aplastic anemia:

1. With general damage to hematopoiesis (acute aplastic anemia, subacute hypoplastic anemia, chronic hypoplastic anemia).

2. With partial damage to erythropoiesis - acquired partial (pure red cell) anemia (autoimmune hemolytic anemia with antibodies against the bone marrow normoblast antigen - according to L.I. Idelson).

Anemia caused by blood loss.

Hemolytic anemia:

A) Hereditary hemolytic anemia:

1) Hereditary hemolytic anemia associated with disruption of the erythrocyte membrane:

Violation of the membrane protein structure (hereditary microspherocytosis, hereditary elliptocytosis, hereditary stomatocytosis, hemolytic anemia associated with the hereditary absence of Rh antigens (Rh-null disease);

Membrane lipid disruption

2) Hereditary hemolytic anemia associated with impaired activity of erythrocyte enzymes:

Hemolytic anemia associated with impaired activity of enzymes of the pentose-phosphate cycle (deficiency of glucose-6-phosphate dehydrogenase activity, deficiency of 6-phosphate dehydrogenase activity);

Hemolytic anemia associated with impaired activity of glycolytic enzymes (pyruvate kinase, triosephosphate isomerase, glucose phosphate isomerase, 2,3-diphosphoglycerate mutase, hexokinase, phosphoglycerokinase, phosphofructokinase).

Hemolytic anemia associated with impaired glutathione metabolism (deficient activity of glutathione synthetase, glutathione reductase or glutathione peroxidase);

Hemolytic anemia associated with impaired activity of enzymes involved in the use of ATP;

Hemolytic anemia associated with impaired nucleotide metabolic activity;

Hemolytic anemia caused by impaired activity of enzymes involved in the synthesis of porphyrins.

3) Hereditary hemolytic anemia associated with a violation of the structure or synthesis of hemoglobin:

Anemia associated with impaired synthesis of globin chains (b-thalassemia, heterozygous, homozygous, bd-thallasemia, ά-thalassemia, homozygous, hemoglobinopathy H, heterozygous intermediate, heterozygous minor, asymptomatic carriage of the ά-thalassemia gene);

Anemia associated with disruption of the structure of globin chains.

B) Acquired hemolytic anemia:

1) Hemolytic anemia associated with exposure to antibodies:

Isoimmune hemolytic anemia ( hemolytic disease newborns, post-transfusion hemolytic anemia);

Autoimmune hemolytic anemia with antibodies against peripheral blood erythrocyte antigens;

2) Hemolytic anemia associated with changes in the structure of the membrane caused by a somatic mutation - Marchiafava-Micheli disease (paroxysmal nocturnal hemoglobinuria).

3) Hemolytic anemia associated with mechanical damage to the membrane of red blood cells (when they come into contact with a prosthetic heart valve or septum, microangiopathic hemolytic anemia - hemolytic-uremic syndrome).

4) Hemolytic anemia caused by chemical damage to red blood cells.

5) Hemolytic anemia caused by a lack of vitamins (vitamin E, etc.).

V. Anemia in various diseases:

A) Anemia in hematological diseases and malignant neoplasms.

B) Anemia due to endocrine diseases.

B) Anemia in burn disease.

This classification is based on the etiopathogenetic principle of grouping anemias, so it is very voluminous and detailed.

For propaedeutics, it is enough to know that anemia can be:

Deficient (with a predominant deficiency of iron, proteins, vitamins).
Posthemorrhagic (due to acute and chronic blood loss).
Hypo- and aplastic (family-hereditary and acquired).
Hemolytic (congenital, family-hereditary and acquired, immune and non-immune).
Symptomatic (against the background of various diseases).

Most common complaints, patients with anemia are indications of pallor of the skin (sometimes with a icteric tinge), fatigue, lethargy, shortness of breath, decreased appetite and motor activity.

From medical history you need to find out:

For deficiency anemia - defects in feeding and care, toxicosis and anemia during pregnancy in the mother, prematurity, frequent and long-term illnesses, especially lesions of the gastrointestinal tract ( chronic gastritis, malabsorption syndrome);

For patients with congenital hemolytic anemia, family-hereditary predisposition is of great importance; for patients with immune hemolytic anemia, incompatibility of the blood of mother and fetus with respect to the Rh factor and group antigens;

For patients with acquired hemolytic anemia, autosensitization factors are important - infections, vaccinations, medications, insect bites;

Chronic blood loss and vitamin B12 deficiency can occur in children and adolescents with erosive gastritis, gastric and duodenal ulcers, in girls with menstrual irregularities.

Clinical picture deficiency anemia characterized by pallor of the skin and visible mucous membranes, lethargy, fatigue, sometimes micropolyadenia (if there is an infection in the child’s body), moderate hepatomegaly. In infants and early age Anemia is often accompanied by other deficiency conditions - rickets, dystrophies, diathesis.

Congenital hypoplastic anemia is characterized by a high degree of stigmatization, and all types of hemolytic anemia are characterized by jaundice and hepatosplenomegaly.

Loss of consciousness is characteristic mainly of severe hemolytic anemia due to bilirubin intoxication and acute renal failure against the background of hemolytic-uremic syndrome.

Changes in the respiratory and cardiovascular systems are secondary and are aimed at compensating for hypoxia due to a decrease in the transport (in relation to oxygen) function of the blood. In addition, they are caused by a decrease in blood viscosity, acceleration of blood flow and dystrophic changes in the myocardium. Therefore, the most common symptoms of anemia are shortness of breath, tachycardia, muffling of the first sound at the apex of the heart, and “anemic” functional murmurs.

In case of hemolytic anemia due to hemolytic-uremic syndrome, symptoms of acute renal failure may appear.

Laboratory diagnostics anemia begins with a general clinical blood test, which makes it possible to:

1. Determine the severity of anemia. At the same time, for deficiency anemias, the determining feature is the degree of decrease in hemoglobin, and for hypoplastic and hemolytic anemias, the number of red blood cells is the determining feature.

Anemia I degree of severity - red blood cells - 3-3.5 × 10 12 / l, Hb - 100-90 g/l;

Anemia of the second degree of severity - red blood cells - 3-2.5 × 10 12 / l, Hb - 90-70 g/l;

Anemia of III degree of severity - red blood cells - less than 2.5 × 10 12 / l, Hb - less than 70 g / l.

It should be remembered that the determining criterion for the severity of anemia is the severity clinical manifestations disease and the intensity of exposure of the patient to risk factors for the development of anemia.

The number of red blood cells is 3.5-4×10 12 / l and the hemoglobin content is 100-110 g / l in children infancy and 4-4.5×10 12 / l and Hb - 110-120 g/l in older children correspond to the concept of latent iron deficiency.

2. Evaluate the color indicator. Normally it is 0.8-1.0. with hypochromia the color index is below 0.8, with hyperchromia it is above 1.0. Iron deficiency anemia is hypochromic and the color index is significantly reduced. In normochromic anemia (due to acute blood loss, deficiency of protein, vitamins, bone marrow hypoplasia), the color indicator remains normal, hyperchromia of erythrocytes is characteristic of anemia that has developed as a result of hemolysis.

3. Determine the regenerative capacity of the bone marrow in response to anemia. Anemia can be normoregenerative with the content of reticulocytes in the peripheral blood in children under 2 years of age no more than 1.5%, and over 2 years of age - no more than 0.6-0.8%, hyperregenerative and hyporegenerative with a content of reticulocytes correspondingly more than 5% and less than 0.3%.

4. Due to a decrease in the number of red blood cells and changes in blood viscosity in anemia, the ESR is increased. In infectious conditions there may also be neutrophilic leukocytosis.

5. Normocytosis and poikilocytosis are signs of a fairly high regenerative capacity of the bone marrow, often combined with hypochromia of erythrocytes. Macrocytosis is usually combined with red blood cell hyperchromia.

To clarify the pathogenesis of anemia and conduct differential diagnosis with other diseases, the following additional studies must be carried out:

Define content serum iron and iron-binding capacity of serum to confirm the iron deficiency nature of anemia;

Define content total protein in blood serum to confirm the protein deficiency nature of anemia (below 55 g/l)

Hemolytic anemia is characterized by the presence of abnormal forms of erythrocytes - microspherocytes, sickle and oval erythrocytes (with familial hereditary Minkowski-Shaffar anemia), high levels in the blood serum indirect bilirubin, altered osmotic resistance of erythrocytes to hypotonic solutions of sodium hypochloride.

To confirm the immune nature of anemia, the Coombs test is performed with red blood cells (if it is immune in nature, it is positive).

Semiotics of hyperplastic syndrome and reactive changes leukograms for leukemia

Modern classifications of acute leukemia are based on the assumption that in malignantly transformed cells the phenotypic characteristics characteristic of the original ones are preserved. normal cells. The main variant of acute leukemia in children is acute lymphoblastic leukemia, which accounts for 75% of all cases of acute leukemia in childhood. Another variant of acute leukemia is acute non-lymphoblastic leukemia, observed in 15-20% of cases. This variant (myeloid leukemia) has a worse prognosis. Other forms of leukemia are characterized as an acute, unidentifiable variant. Separately, congenital leukemia is distinguished, which occurs through different options and is a special form.

Main complaints patients with acute leukemia, along with complaints characteristic of patients with anemia, are fever, weight loss, hemorrhagic rash on the body, ulcers on the skin and mucous membranes, bleeding, bone pain.

The history of patients with leukemia does not always allow one to suspect the cause of the disease. There are indications of hereditary predisposition, unfavorable environmental conditions (high levels of radiation), previous infectious diseases and injuries.

The disease often begins unnoticed. The initial period often occurs under the guise of an acute respiratory disease, allergic condition, etc. Then the disease enters acute period. Its clinical picture is polymorphic; leukemia has many “masks.” It is advisable to identify a number of syndromes.

1. Intoxication syndrome is manifested by a complex of asthenovegetative disorders, lethargy, weakness, gray-icteric, earthy-greenish skin tone, temperature reactions, often in the form of low-grade fever.

2. Anemic syndrome is characterized by waxy pallor of the skin, the appearance of a “spinning top noise” upon auscultation of the heart. In acute leukemia, all germs of hematopoiesis are inhibited, including erythrocytes, therefore hypoplastic anemia develops, characterized by a decrease in the number of reticulocytes and with normochromia of erythrocytes.

3. Proliferative syndrome is especially characteristic of acute lymphoblastic leukemia. There is an increase in lymph nodes, both peripheral and abdominal and mediastinal, and hepatosplenomegaly. Hepatolienal syndrome in leukemia is caused by the presence of leukemic foci in the liver and spleen, toxic damage to the liver, and increased load on the spleen due to the massive decay of a large number of immature forms of leukocytes. Hepatolienal syndrome is especially pronounced in myeloid leukemia, and to a lesser extent in lymphoblastic leukemia. The liver and spleen are dense, their lower edge can reach the iliac crest. The pain is caused by overstretching of the capsules. Symmetrical increase in salivary and lacrimal glands defined as Mikulicz syndrome. At the same time, the child characteristic appearance- puffy face, periorbital edema, lacrimation. “Leukemids” can be found on the skin and mucous membranes - external manifestations leukemic infiltration. Typical symptoms include urinary tractitis, stomatitis, and sore throats, often necrotic.

4. Cutaneous hemorrhagic and ulcerative necrotic syndromes in leukemia are caused by thrombocytopenia due to inhibition of the platelet lineage of hematopoiesis. The rash is petechial-spotty in nature. On the skin and mucous membranes of the patient, petechiae, ecchymosis are detected, spontaneous bleeding and bleeding that occurs at the slightest injury are possible (nasal, gum, uterine, kidney), elements have different sizes and are in various stages flowering. Hematomas are rare. Due to toxic damage to the walls of blood vessels, a vasculitic purpuric type of skin hemorrhage is also possible. It is on the basis of these papular-hemorrhagic elements, as well as as a result of secondary infection of injuries to the skin and mucous membranes, that necrosis and ulcers are formed, including on the mucous membranes of the respiratory tract and gastrointestinal tract.

5. Osteoarticular syndrome is characterized by pain in the bones and joints, which are sometimes very intense due to the presence of leukemic infiltrates.

6. Syndrome neurological disorders is caused, first of all, by true neuroleukemia, that is, the presence of leukemic foci in the central nervous system. It develops gradually and often manifests itself in remission, interrupting its course. Neurological symptoms of neuroleukemia are very diverse: headaches, vomiting, meningeal symptom complex due to intracranial hypertension, focal symptoms in the form of the development of paralysis and paresis, disorders of cranial innervation, a radicular symptom complex is possible. Neuroeukosis is one of the most formidable and prognostically unfavorable manifestations of leukemia.

In addition to the syndromes listed above, leukemia may cause secondary changes in the respiratory system (pneumonia), in the cardiovascular system (rhythm disturbances, arterial hypertension from hormone therapy), from the gastrointestinal tract (intestinal bleeding, necrotizing ulcerative enterocolitis) and kidneys (hematuria, toxic nephritis).

Laboratory diagnosis of leukemia includes a general blood test, myelograms and cytochemical reactions of blast cells to determine the type of leukemia.

In a general clinical blood test for acute leukemia, we will see:

Anemia, often severe, normochromic, hyporegenerative;

Thrombocytopenia, increased bleeding duration and impaired blood clot retraction;

On the part of white blood against the background of leukopenia, leukemic gaping is characteristic, that is, the absence of transitional forms of maturing leukocytes between blast cells and mature leukocytes;

The ESR is sharply increased.

In chronic leukemia, anemia is less pronounced, there may be no thrombocytopenia, leukopenia is rare, on the contrary, leukocytosis is usually pronounced. Leukogram without leukemic dehiscence, it presents all stages of leukocyte maturation.

The final diagnosis of leukemia and determination of its variant is carried out after examining bone marrow puncture. The myelogram reveals up to 90-95% of blast cells, but an increase in blasts of more than 25% already makes one think with certainty about acute leukemia. In the myelogram, as in the peripheral blood, acute leukemia is characterized by the absence of transitional forms between blasts and mature leukocytes.

The leukemia variant is determined using cytochemical reactions. Lymphoblasts, which are the substrate of acute lymphoblastic leukemia, are characterized by a granular arrangement of glycogen in at least 10% of blast cells. All blasts in acute lymphoblastic leukemia do not contain myeloperoxidase or lipids. Blasts in myeloid leukemia are characterized by a diffuse arrangement of glycogen. The reaction to myeloperoxidase and lipids is positive.

Hemorrhagic diathesis.

Semyotka cutaneous hemorrhagic syndrome.

The term “hemorrhagic diathesis” unites diseases of different etiology and pathogenesis that have a common clinical syndrome - bleeding.

According to the tradition established in pediatrics, hemorrhagic diathesis is divided into three main groups:

Vasopathies are diseases that primarily affect the walls of blood vessels.
Thrombocytopenia and thrombocytopathy are diseases with disruption of the platelet component of hemostasis.
Coagulopathies are diseases with impaired blood clotting, the vast majority of which are caused by a deficiency of coagulation factors VIII or IX - hemophilia A and B.

Semiotics of hemorrhagic syndrome:

Blood diseases are characterized by skin elements of a non-inflammatory nature. They are formed due to hemorrhages in the skin or mucous membranes. Depending on the pathogenesis of the disease and the predominant nature of skin hemorrhages, the following types of bleeding are distinguished:

Thus, for a patient with hemophilia, the most typical complaints are prolonged bleeding from the slightest injury, repeated hemarthrosis affecting 1-2 large joints. The history provides clear indications of a family-hereditary predisposition to bleeding in men.

Clinically, hemophilia is distinguished:

A - the most common hereditary coagulopathy, caused by factor VIII deficiency;

B - is caused by a deficiency of coagulation factor IX, is more rare than hemophilia A, and has a similar clinical course.

C - factor XI deficiency. An even rarer coagulopathy. Its course is usually mild, bleeding occurs only after injuries and surgical interventions. Hemarthrosis and cerebral hemorrhages usually do not occur. Both boys and girls get sick.

Clinical manifestations of hemophilia in a newborn child (bleeding from the remnant of the umbilical cord) are relatively rare, since due to maternal blood clotting factors received by the child antenatally and after birth with breast milk, hemostasis in the child is preserved in the first months of life. For the first time, bleeding in such children is detected during teething, injections during preventive vaccinations and when expanding physical activity with the risk of injury. The type of bleeding is hematoma. Hemarthrosis leading to ankylosis of the joint is typical.

Thrombocytopenic purpura (Werlhof's disease) can be family-hereditary, congenital, immune and non-immune.

Classification of thrombocytopenias

Most common complaint patients suffer from spontaneous bleeding in the form of nasal, gingival, uterine and other bleeding and hemorrhagic skin rash.

It is important to find out information about their anamnesis about bleeding or splenectomy in relatives, about hemorrhagic syndrome in a patient in the neonatal period, about infections preceding hemorrhage, vaccinations, and medications.

Cutaneous hemorrhagic syndrome of petechial-spot type. Due to the polymorphism of the rash in terms of time of occurrence and size, it is often compared to “leopard skin.” Most frequent sight bleeding is from the nose. Girls at puberty experience uterine bleeding. The favorite localization of the rash is the back, buttocks, and thighs. hepatolienal syndrome is characterized by a predominant enlargement of the spleen.

Hemorrhagic vasculitis- has an immunopathological nature.

The following clinical forms of hepatitis B are distinguished: simple (isolated skin purpura), skin-articular, mixed (cutaneous-abdominal, skin-articular-abdominal), with visceral lesions (nephritis, cardiopathy). The course of the disease can be acute, lightning fast, protracted (wavy), chronic (recurrent).

Main complaints patients with hemorrhagic vasculitis are intoxication, low-grade fever, the appearance of a hemorrhagic rash on the skin, joint pain, vomiting with blood, abdominal pain, the appearance of blood in the urine and feces.

IN medical history patients there are indications of the presence of foci chronic infection, allergic mood in the child, acute infectious diseases or preventive vaccinations suffered 2-3 weeks before the onset of hemorrhagic syndrome.

Isolated cutaneous purpura is characterized by a vasculitic purpuric type of bleeding.

Skin-articular syndrome is manifested by arthralgia or arthritis, mainly of large joints. Arthritis and arthralgia are of a volatile nature and do not lead to joint deformation.

Abdominal syndrome is caused by hemorrhages in the mucous membrane of the gastrointestinal tract and is clinically manifested by abdominal pain, vomiting with blood and discoloration feces(black feces or an admixture of light blood, depending on the level of damage).

Renal syndrome develops when the capillaries of the renal glomeruli are damaged and is clinically manifested mainly by macro- or microhematuria.

Laboratory examination of patients with hemorrhagic diathesis begins with general analysis blood, which reveals the following changes:

All hemorrhagic diathesis are characterized by posthemorrhagic anemia, normochromia, normo- or hyperregenerative anemia. However, in the future, due to the depletion of the compensatory capabilities of the bone marrow, anemia may become hyporegenerative.
Moderate leukocytosis or normal leukogram values are characteristic of hemophilia and thrombocytopenic purpura. With hemorrhagic vasculitis, due to its infectious-allergic nature, moderate reactive leukocytosis with a non-hytrophilic shift in the leukocyte formula is characteristic.
Acceleration of ESR can be caused by anemia, or in hemorrhagic vasculitis by the infectious-allergic nature of the disease. Therefore, ESR is especially sharply increased in vasculitis.
Thrombocytopenic purpura is characterized by: thrombocytopenia, increased duration of bleeding, normal time blood clotting, decreased or complete absence of blood clot retraction.
For hemophilia, the main indicator is a slowdown in blood clotting time with normal platelet counts, blood clot retraction and bleeding duration.

To differentiate hemophilia from other coagulopathies, a detailed coagulogram is examined. To clarify the immune or non-immune nature of thrombocytopenic purpura, the Coombs reaction is performed with platelets.

“Reactive” changes in the leukocyte formula

Characteristic of physiological leukocytosis of a newborn child , reactive inflammatory leukocytosis, eosinophilic-leukocyte reactions.

Reactive inflammatory leukocytosis in microbial inflammatory diseases is manifested by an increase in the number of leukocytes in the peripheral blood, combined with a shift in the leukocyte count to the left due to band and segmented neutrophils. Sometimes there may be a rejuvenation of the leukogram to young, myelocytes and metamyelocytes. The described picture is typical for sepsis, pneumonia, pyelonephritis. In infants and young children with infectious inflammatory diseases, there may be not an increase, but a decrease in the number of leukocytes due to inhibition of leukopoiesis under conditions of infectious toxicosis, but a shift in the leukocyte formula to the left always remains. Reactive leukocytosis is often combined with anemia, progressive in the dynamics of the disease and accelerated ESR.

In diseases of a microbial-inflammatory nature, changes in the leukocyte formula can be represented in the form of three successive phases:

1) In the acute phase, neutrophil defense predominates (shift of the formula to the left);

2) When acute phenomena subside, the number of leukocytes and neutrophils decreases and the number of monocytes increases (monocyte phase of protection);

3) During the period of convalescence, the number of lymphocytes and eosinophils increases (lympho-eosinophilic protection)

Among other changes in white blood, it is necessary to remember the toxic granularity of neutrophils and neutropenia, characteristic of severe sepsis, poisoning, and radiation injuries.

Reactive leukocytosis has some features in infectious and allergic diseases (rheumatism, glomerulonephritis, hemorrhagic vasculitis). Usually, in these diseases, moderate leukocytosis (10-12 ×10 9 /l), neutrophilia, eosinophilia and a sharply accelerated ESR are observed.

At viral diseases leukopenia with lymphocytosis is often observed. With mononucleosis, atypical mononuclear cells appear in the blood.

In children with an allergic predisposition against the background of viral infections, during exacerbations bronchial asthma, atopic dermatitis there may be an eosinophilic-leukemoid reaction, manifested by an increase in eosinophils, the appearance of basophils and plasma cells in the peripheral blood. Plasma cells can appear in the blood and in viral diseases