The main hematological syndromes in diseases of the hematopoietic system. Diseases of the hematopoietic organs

Blood diseases in adults are considered one of the most formidable, as they develop extremely rapidly and are severe, damaging various systems and organs. A person is able to independently suspect a progressive pathology, but it is impossible to differentiate it without a specialist.

The greatest danger in the course of blood diseases is the difficulty of early diagnosis, since most of the symptoms are not specific to this nosological group, and different kinds The patient most often attributes ailments to overwork, seasonal vitamin deficiency and considers it a transient phenomenon. In the meantime, the disease continues to progress, and the lack of treatment can be fatal.

A disorder of the hematopoietic system can be assumed by the following signs:

increased fatigue, drowsiness, not related to the load during the day, psycho-emotional state and quality of rest;
change in the skin - depending on the diagnosis, the skin and mucous membranes may become pale, gray, or covered with a hemorrhagic rash;
dry skin and mucous membranes, hair loss, brittle nails;
dizziness, weakness;
night sweats;
increase lymph nodes;
the appearance of spontaneous bruising;
an increase in body temperature without a clinic of a respiratory viral disease;
bleeding gums, may be nosebleeds.

To make a diagnosis, it is necessary to conduct laboratory tests, which will include a clinical and biochemical blood test, a coagulogram taking into account the values of RFMK and d-dimer (according to indications), and additional pathological markers such as homocysteine, antiphospholipid antibodies, C -reactive protein, some antigens, thromboelastogram, coagulation factors and platelet aggregation.

Classification of blood diseases:

The key point in the development of the disease is pathology at one of the levels of hematopoiesis.

The range of diseases that can be identified include:

Anemia:

deficiency anemia (iron deficiency, B12 deficiency, folic acid deficiency);
hereditary dyserythropoietic anemia;
posthemorrhagic;
hemolytic;
hemoglobinopathy (thalassemia, sickle cell anemia, autoimmune, etc.);
aplastic anemia.

Hemorrhagic diathesis:

hereditary coagulopathy (hemophilia, von Willebrand disease, rare hereditary coagulopathy);
acquired coagulopathy (hemorrhagic disease of the newborn, deficiency of K-vitamin-dependent factors, DIC);
disorders of vascular hemostasis and mixed genesis(Rendu-Osler disease, hemangiomas, hemorrhagic vasculitis, etc.);
thrombocytopenia (ideopathic thrombocytopenic purpura, alloimmune purpura of newborns, transimmune purpura of newborns, heteroimmune thrombocytopenias);
thrombocytopathy (hereditary and acquired).

Hemoblastoses:

myeloproliferative diseases;
myelodysplastic diseases;
myelodysplastic syndromes;
acute myeloid leukemia;
B-cell neoplasms;
Histiocytic and dendritic cell neoplasms

Pathologies circulatory system characterized by a change in the number of blood elements, their quality, structure and shape with a parallel decrease in their functions. Diagnosis is quite difficult, since the deviation from normal indicators blood, can be in almost any other disease of the body. Diagnosed disease requires immediate medical intervention and changes in diet.

DIC

Disseminated intravascular coagulation develops as a result of concomitant pathology, which stimulates the organs of the circulatory system to hypercoagulation. long current acute stage DIC leads to complete destabilization of hemostasis, where hypercoagulation is replaced by critical hypocoagulation. In this regard, therapy varies depending on the stage of the disease - at one stage, anticoagulants and antiplatelet agents will be used, while the other stage may require blood transfusion.

Disseminated intravascular coagulation is accompanied by general intoxication, weakness, dizziness, impaired thermoregulation.

DIC can be triggered by:

acute bacterial infection;
violation of the gravid period caused by fetal death, placental abruption, eclampsia, amnion embolism;
serious injury;
tissue necrosis;
organ transplantation, transfusion;
acute radiation sickness, hemoblastosis.

Treatment of the syndrome is aimed at stabilizing the coagulation and anticoagulation system, neutralizing blood clots and microclots, restoring adequate function and platelet count with normalization of the APTT time. The laboratory criteria for the success of therapy is considered to be the entry into the reference values of d-dimer, APTT, RFMK, fibrinogen and platelet count.

Anemia

One of the types of anemia can be found in every fourth person on Earth, and most often it is caused by a deficiency of vitamins or trace elements. Anemia is a disease in which either the number of erythrocytes in the plasma decreases, or the hemoglobin content inside the erythrocytes decreases. Pathology may owe its development to either a poor-quality diet, or damage to the hematopoietic organs, or massive blood loss, in which the level of hemoglobin in the blood cannot be restored to normal after bleeding. There are also other types of anemia, less common, but more formidable (genetic, infectious).

To diagnose anemia, as well as to clarify its type, it is necessary to assess the level of hemoglobin, the number of erythrocytes, hematocrit, the volume of erythrocytes, the average concentration of hemoglobin in the erythrocyte.

Anemia caused by helminthic invasion requires not only anthelmintic treatment, but also the use of a complex of vitamins to eliminate beriberi.

To clarify the nature of anemia, tests are prescribed to assess the level of trace elements in the blood - the amount of cyanocobalamin is considered, folic acid and iron in plasma. With a lack of one or another component, a medical preparation is prescribed and nutrition is adjusted.

Video - Anemia: how to treat

Thrombophilia

Thrombophilia is a group of diseases in which the blood coagulation system is excessively activated, which causes the pathological formation of clots and blood clots. Thrombophilia can be acquired - such as antiphospholipid syndrome, as well as congenital or genetic - in the presence of active (worked) mutations in hemostasis genes. Presence of predisposition - detected mutant genes, high level homocysteine, the presence of antiphospholipid antibodies is a significant risk factor for the development of thrombosis of various localization.

The risk of thrombosis increases significantly if, in the presence of a predisposition, there is a habit of smoking, overweight, folate deficiency, oral contraceptives are taken, and a sedentary lifestyle is maintained. In pregnant women, the risk of thrombosis in the presence of mutations in hemostasis genes is even higher, in addition, the likelihood of fetal loss at any gestational age increases.

Depending on the type of thrombophilia, it is possible to prevent the development of pathology by taking folic acid and other B vitamins, maintaining active image life, exclusion of use oral contraception, control of hemostasis in preparation for pregnancy and during it. Prophylactic doses of antiplatelet agents and anticoagulants may also be needed - it all depends on the actual situation and history.

To diagnose thrombophilia, the doctor prescribes:

hemostasis genes: F2, F5, PAI-1, fibrinogen;
folate cycle genes, homocysteine;
antibodies to phospholipids, cardiolipin, glycoprotein;
lupus anticoagulant;
hemostasiogram with RFMK and d-dimer.

Thrombophilia can be expressed in thrombosis of the veins of the lower extremities, thrombophlebitis, hyperhomocysteinemia, thromboembolism, in pregnant women - gestosis and eclampsia, sclerosis and thrombosis of the chorionic villi, which leads to fetal hypoxia, oligohydramnios and even death of the fetus. If pregnant women with a burdened obstetric history have never experienced thrombosis, then antiplatelet agents may be prescribed to increase the chances of gestation, since this group of patients has excessive aggregation of blood clots from the first trimester.

But hemophilia is a completely opposite disease, and its severe forms, as a rule, end in failure. Hemophilia includes a group of hereditary diseases in which there is a mutation of coagulation genes, which leads to a high risk of developing bleeding with a fatal outcome.

Thrombocytopenia and thrombocytopenia

Thrombocytopenia can be either an independent disease due to disruption of the bone marrow or spleen, or provoked by taking anticoagulant drugs. Thrombocytopenia is characterized by a decrease in the number of platelets. If a this pathology appeared against the background of taking heparin, especially in the first 15 days from the start of therapy, it is urgent to cancel the drug. Most often, this complication is caused by sodium heparin, therefore, with this kind of anticoagulant treatment, control over the number of platelets, the level of antithrombin 3 and APTT is required to avoid bleeding.

As an independent disease, thrombocytopenia acts as purpura, often of a congenital and autoimmune nature. For treatment, drugs that stabilize hemostasis, as well as drugs that help immune activity, are used.

Thrombocytopathy can act as a hereditary disease with mild symptoms that can be treated with vitamin supplements and dietary changes.

In the case of thrombocytopenia, an adequate amount of blood cells is produced, but they have an altered structure and have inferior functionality. Most often, thrombocytopathy is caused either by taking drugs that thin the blood, or by a violation of the bone marrow. Given the disease, the aggregant ability of platelets and their adhesion are disturbed. Treatment is aimed at reducing blood loss by taking vitamins and aggregates.

Less common blood disorders

There are also blood pathologies that are many times less common than anemia, DIC, and thrombocytopenia. This reduced frequency is related to the specificity of the diseases. Such pathologies include:

genetic disease thalassemia with impaired hemoglobin production;
malaria with the destruction of erythrocyte mass;
leukopenia, neutropenia - significant pathological decline the number of leukocytes - most often acts as a complication of the underlying disease;

agranulocytosis, which develops against the background of an autoimmune reaction;
polycythemia - a sharp abnormally high increase in the number of red blood cells and platelets;
oncological lesions of the blood - leukemia or leukemia, hemoblastoses;
sepsis is a well-known acute infectious disease, commonly known as blood poisoning.

When clarifying the diagnosis, it should be remembered that one blood disease can gradually transform into another (leukopenia with the progression of lupus erythematosus syndrome can develop into agranulocytosis), and can also be not an independent phenomenon, but a complication or a sign of a certain pathological process.

Search disease state according to blood tests - a very grateful thing, as it allows you to confirm or exclude serious illnesses circulatory system. Even if hemostasis is within the normal range, but the overall clinical analysis indicates the current pathological process, then the search for the source of violations is greatly facilitated. The symptoms of blood diseases in an adult are very non-specific and can be easily mistaken for signs of another disease, so the study of the main hematological parameters should be the starting point for eliminating the disease.

To blood system relate hematopoietic organs (Bone marrow, lymph nodes, spleen, liver) and the blood itself with formed elements, plasma and chemicals.

The main hematopoietic organ is the red bone marrow, in which the ancestral nuclear stem cells are formed - blasts . Formed elements are sequentially formed from blasts, mature and are released into the peripheral blood: erythrocytes, leukocytes and platelets. Erythrocytes are produced mainly in the red bone marrow, leukocytes - in the spleen and lymph nodes (one of the forms of leukocytes is produced in the spleen - monocytes, in the lymph nodes - lymphocytes), platelets - in the red bone marrow.

Blood supplies nutrients to all tissues of the body and removes harmful products. blood plasma - clear liquid that remains after removal shaped elements from blood. Plasma consists of water with protein substances dissolved in it, sugar, the smallest particles of fat, and various salts. The amount of blood in the human body is 5-5.5 liters.

Formed elements of blood:

Erythrocytes. Norm: for men: 4.0 - 5.5 x 10 12 / l. For women: 3.7 - 4.7 x 10 12 / l.

Red blood cells are evaluated in a smear. Pay attention to their size, shape, color and cellular inclusions. Normal erythrocytes have round shape. With anemia different nature the size of erythrocytes changes. The appearance of red blood cells of different sizes is called anisocytosis. The predominance of small erythrocytes - microcytosis- characteristic of iron deficiency anemia, macrocytosis- with B 12 deficiency anemia. Under pathological conditions of maturation of erythrocytes, a change in the shape of erythrocytes is noted - poikilocytosis. Increase in the number of red blood cells (erythrocytosis) can be absolute or relative. The cause of absolute erythrocytosis is a reactive irritation of erythropoiesis with an increase in the mass of circulating erythrocytes in congenital and acquired heart defects, pneumosclerosis, some tumors: kidney cancer, pituitary adenoma, and relative erythrocytosis is blood clotting without an increase in erythropoiesis during shock, burns, a rapid increase in edema, diarrhea, profuse vomiting. At erythremia the number of erythrocytes increases and reaches 9.0 - 12.0 x 10 12 /l.

Reducing the number of red blood cells - erythrocytopenia. It may be the result of increased breakdown of red blood cells in hemolytic anemia, vitamin B12 deficiency, iron, bleeding, insufficient protein content in food, leukemia, multiple myeloma, metastases of malignant tumors, etc.

Red blood cells give blood a red color, as they contain a special substance - hemoglobin. The norm for men: 130 - 160 g / l, for women: 120 - 140 g / l.

Increase in hemoglobin observed with thickening of the blood after vomiting, with burns, severe diarrhea, various intoxications, erythremia, some birth defects hearts. Decrease in hemoglobin noted with iron deficiency and hemolytic anemia, after blood loss, with a deficiency of vitamin B12 and folic acid. Erythrocytes, passing through the lungs with blood, capture the oxygen of the air and carry it to all organs and tissues.

The lifespan of an erythrocyte is 120 days; they are destroyed most of all in the spleen. Hemoglobin, released after the destruction of red blood cells, is an integral part of the bilirubin formed by the liver, iron is used to build new red blood cells.

Availability reticulocytes(immature forms of erythrocytes, the norm is up to 1% in the blood) in peripheral blood and in the bone marrow (true reticulocytosis) is an indicator of the regenerative capacity of the bone marrow. An increase in them is noted with hemolytic anemia, blood loss, malaria, polycythemia, and during the treatment of iron deficiency and B 12 deficiency anemia.

Leukocytes. Leukocytes are capable of active movement, they can absorb substances foreign to the body, such as dead cells (phagocytosis). They play an important role in protecting the body from germs.

Leukocytes, unlike erythrocytes, contain a cell nucleus. The blood normally contains 4-9 10 9 /l of leukocytes. There are granulocytes (granular - with granular protoplasm) and agranulocytes (non-granular) leukocytes. Granulocytes are eosinophils, basophils, neutrophils. Agranulocytes are lymphocytes and monocytes.

Towards total number of leukocytes, neutrophilic granulocytes make up about 50-70%, of which myelocytes are not normally detected in peripheral blood, young up to 1%, stab up to 5%, segmented 51-67%; lymphocytes - up to 30%, monocytes - up to 8%, eosinophils - 2-4%, basophils - 0.5-1%.

An increase in the number of leukocytes ( leukocytosis) is noted in acute inflammatory and purulent processes, poisoning and acute infectious diseases, with the exception of most viral infections, with closed skull injuries, cerebral hemorrhages, diabetic and uremic coma, in the first days after myocardial infarction, in the primary reaction of acute radiation sickness. Leukocytosis in acute and chronic leukemia reaches hundreds of thousands -100.0 x 10 9 /l and more.

Decrease in the number of leukocytes ( leukopenia) is observed during radiation sickness during the peak, viral diseases(Botkin's disease, influenza, measles), systemic lupus erythematosus, hypo- and aplastic anemia, aleukemic variants of acute leukemia, after taking various drugs (sulfonamides).

Eosinophilia(increase in the content of eosinophils) is often observed in helminthiases, with allergic diseases(bronchial asthma, dermatoses, etc.); with collagenoses (rheumatism, etc.); with burns and frostbite. eosinopenia(decrease in the content of eosinophils) occurs in typhoid fever, viral hepatitis and other infectious and viral diseases.

Shift leukocyte formula to the left is an increase in the number of stab, young and the appearance of myelocytes, occurs with tonsillitis, acute appendicitis, lung abscess, active tuberculosis, severe forms of pneumonia, diphtheria, sepsis, purulent meningitis, acute cholecystitis, peritonitis, with leukemia and leukemoid reactions. With leukemia and leukemoid reactions, there may be myelocytes, promyelocytes and myeloblasts in the peripheral blood.

lymphocytosis indicates a favorable course of the disease in pneumonia, erysipelas, diphtheria, tuberculosis and others chronic infections.Lymphopenia observed in lymphogranulomatosis.

Monocytosis observed in diphtheria, rubella, scarlet fever.

Basophilia seen in diabetes mellitus acute hepatitis with jaundice, in radiologists exposed to long-term exposure to low doses; with hypothyroidism, lymphogranulomatosis, chronic myeloid leukemia. The number of basophils decreases with tuberculosis, hyperthyroidism, after X-ray therapy, with acute leukemia.

platelets (platelets) are involved in the process of blood clotting. Normally, they contain 180-320 10 9 / l. An increase in the number of platelets - thrombocytosis observed at posthemorrhagic anemia, erythremia, chronic myeloid leukemia, malignant tumors, atrophy of the spleen of various etiologies, after removal of the spleen. Thrombocytopenia characteristic of idiopathic thrombocytopenic purpura (Werlhof's disease), radiation sickness, hypo- or aplastic anemia, acute leukemia, hypersplenism, lead poisoning, benzene, chronic nephritis.

ESR. Norm: For women: 2-15 mm / h. For men: 2-10 mm/h.

ESR is not a specific indicator for any disease, but the acceleration of ESR indicates the presence of a pathological process. It rises 24 hours or a few days after the onset with any inflammatory process and infectious disease (with purulent-septic processes, parenchymal liver lesions, collagenoses, including rheumatism), as well as with anemia, after myocardial infarction, blood transfusion. After the disappearance of clinical signs, ESR slowly returns to normal.

Diagnosis of diseases of the blood system.

Complaints hematological patients are very diverse and correspond to clinical syndromes :

- anemic syndrome : persistent headaches, dizziness, tinnitus, shortness of breath, feeling of "lack of air", palpitations, pain in the heart, fainting, increased fatigue and irritability, memory loss, "flies before the eyes", objectively manifested by pallor skin and mucous membranes, it is possible to detect an increase in respiration and pulse, systolic murmur over all points of auscultation of the heart and a "top" noise over large veins (due to accelerated blood circulation and a decrease in blood viscosity due to erythrocytopenia), a moderate decrease blood pressure, pastosity of the lower extremities. Complaints are associated with cell hypoxia and impaired tissue metabolism, manifested in anemia, leukemia, blood loss. Trophic changes skin (thinning, dryness, hair loss, brittle nails) are associated with a lack of iron-containing enzymes in the body

- hemorrhagic syndrome: hemorrhagic manifestations on the skin: hemorrhages in the skin, muscles, joints, at the injection site, petechial rash, bruising of all sizes and stages; bleeding (nasal, gingival, uterine, intestinal). Their causes: thrombocytopenia, platelet deficiency, increased permeability vascular wall, intravascular coagulation.

- ulcerative necrotic syndrome: sore throat, impaired swallowing, salivation, bloating, cramping abdominal pain, mushy stools, lesions of the mucous membranes (aphthous stomatitis and necrotic tonsillitis, esophagitis). Symptoms occur due to a sharp decrease or complete disappearance of granulocytes from the blood, as well as with leukemic growth in the mucous membranes.

- lymphadenopathy: swollen lymph nodes and changes in the configuration of the neck, inguinal, axillary zone. Persistent dry cough and shortness of breath during exercise can be observed with an increase in mediastinal lymph nodes; a feeling of fullness in the abdomen, bloating, flatulence and unstable stools, intestinal obstruction - with an increase in mesenteric and retroperitoneal lymph nodes. In diseases of the blood system, the lymph nodes are often dense, painless, they slowly but steadily increase in size. For lymphadenopathy of inflammatory origin, the soreness of enlarged nodes, the formation of fistulas, the phenomena of general intoxication (fever, chills, neutrophilic leukocytosis, etc.), the disappearance of signs of lymphadenitis as they subside inflammatory process. Significant splenomegaly and hepatomegaly lead to visible enlargement of the abdomen, which is characteristic of some variants of chronic leukemia ( chronic myeloid leukemia, lymphocytic leukemia, etc.) Enlargement of the liver in hematological patients occurs most often due to its acute dystrophy or toxic-allergic hepatitis in acute leukemia, the growth of leukemic tissue in them in chronic leukemia. Enlargement of the spleen in hematological patients is the result of intense hemorrhage (for example, with hemolytic anemia), tumor growth in it (for example, with leukemia, lymphogranulomatosis, etc.).

- intoxication syndrome: weakness (observed in almost all patients in the advanced stage of leukemia due to tissue hypoxia and intoxication of the body with leukemic cells). Sweating (and more often expressed in daytime, which distinguishes it from chronic inflammatory diseases), shortness of breath with physical activity, palpitations, anorexia, weight loss up to cachexia. Skin itching initially occurs after taking a bath, but later becomes painful and constant - caused by the breakdown of cells in the capillaries of the skin with impaired microcirculation, thrombosis and the release of histamine-like substances, sometimes combined with pain in the tips of the fingers and toes. Persistent fever: combined with sweating and due to pyrogenic action products of mass decay of erythrocytes, leukocytes, and other blood cells.

- osteoarthropathic syndrome: pain in the bones (ossalgia) and joints (arthralgia), their swelling, hyperemia of the skin over them, dysfunction of the joint (arthropathy). Arthralgia is sometimes the only symptom, so the bone marrow should be examined. Ossalgia occurs in the vertebrae, ribs, sternum, ilium, less often in the tubular and bones of the skull. They are well identified with pressure on the bone or light tapping on it.

- immunodeficiency syndrome: frequent colds, infectious complications(pneumonia, bronchitis, pyelonephritis, pyoderma) - arise due to the defectiveness of the immune system, when antibodies are produced against normal body cells.

Disease history. Finding out the question of how the patient fell ill, it is necessary to ask in detail about the general condition of the patient that preceded the disease, as well as the alleged factors that provoked the disease. It is necessary to study the dynamics of each symptom, whether there was a blood test, and what were its results; find out what was treated and with what effect.

Anamnesis of life:

It is important to clarify the permanent place of residence, since the unfavorable ecological situation is a risk factor for hematological diseases.

It is necessary to find out the factors that are important in the etiology of the disease: improper, one-sided nutrition, insufficient exposure to fresh air, acute and chronic intoxication in the production of mercury salts, lead compounds, phosphorus, etc.; radiation injury; information about previous diseases that can be complicated by blood diseases - stomach and duodenal ulcers (anemia), diseases with hemorrhagic syndrome, kidney failure.

The question of heredity is great importance in the diagnosis of hemophilia, hereditary anemia.

Taking medications (levomycetin, amidopyrine, cytostatics). To rule out drug damage, ask for a list of all medications the patient has taken in the last 3-4 weeks.

Methods objective examination patients:

inspection data:

Paleness of the skin with a different shade (chlorosis or a greenish tint - with iron deficiency, jaundice - with hemolytic anemia). With erythremia - "full-blooded", cherry-red color of the skin of the face, neck, hands. Examining the skin, one can detect hemorrhages in the form of spots of various sizes and shapes - from small punctate (petechiae) to larger ones (purpura, ecchymosis); the largest hemorrhages are called bruises. Attention should be paid to the state of trophism of the skin. With iron deficiency anemia, the skin is dry, flaky, the hair is brittle, split.

Examination of the oral cavity confirms ulcerative necrotic syndrome, leukemic infiltration (swelling of the gums, enlargement of the tonsils).

An increase in the abdomen occurs with hepato- and splenomegaly (enlargement of the liver and spleen).

Decrease in body weight, scratching on the skin, fever confirm the intoxication of the body.

Deformation and increase in the size of the joints with hemorrhage (hemophilia). Swelling and limitation of movement in the joints are often asymmetrical. Sometimes with blood diseases, fingers are observed in the form of " drumsticks” with nails in the form of “watch glasses”.

Increased respiration and pulse rate, systolic noise over all auscultation points, lowering blood pressure, pastosity of the lower extremities, "top noise" over large veins due to accelerated blood circulation and reduced blood viscosity.

Palpation. If leukemia is suspected, the skeletal system is carefully examined: pressure on flat bones or epiphyses of tubular bones, tapping on them is painful.

Valuable information is provided by palpation of the lymph nodes and spleen. With lymphocytic leukemia and lymphogranulomatosis, systemicity is characteristic, the multiplicity of lesions of the lymph nodes - if one group is affected, then the defeat of other groups is added. Normally, the spleen is not palpable. It becomes accessible to palpation with a significant increase (splenomegaly).

Research of a hemorrhagic syndrome. Capillary permeability is checked by a tourniquet, a pinch, a jar test (the appearance of petechiae is normally observed after 3 minutes distal to the tourniquet).

Percussion. Used to define the boundaries of the spleen.

Auscultation. With anemia, a systolic murmur is heard over the heart and blood vessels, which is associated with a decrease in blood viscosity and an increase in the speed of blood movement in the vessels.

Laboratory research methods.

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 with physical effort), tinnitus, dizziness, weakness, increased fatigue , syncope, etc. From a hematological point of view, anemia can be divided into hypo- and hyperchromic and regenerative and regenerative.

With 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). With hyperchromic anemia, the amount of hemoglobin is lowered less than the number of erythrocytes, and therefore the color index is greater than one (pernicious anemia, hemolytic anemia).

In case of regenerative or aplastic anemia in the peripheral blood (and in advanced cases in the bone marrow) there are no signs of red blood regeneration, i.e., there are no reticulocytes, polychromatophiles, macrocytes, erythroblasts, etc. Since the bone marrow suffers as a whole, then both leukopenia (granulocytopenia) and thrombocytopenia are simultaneously observed, and a picture of the 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 a pronounced pallor of patients in the absence of a decline in nutrition, the presence of gastric achylia and changes in nervous system (spinal cord). Main hematological features pernicious anemia the following: a very sharp decrease in the number of erythrocytes (often less than 10% of their 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 polycythemic syndrome. It is based on increased erythropoiesis. Clinically, it is expressed mainly by disturbances in the activity of the circulatory system: a sharp increase to a dark cherry-red color of the skin of the face and hands, and especially the mucous membrane of the lips and oral cavity and pharynx, 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 erythrocytes per 1 mm3 of blood (up to 12-14 million), clear signs of regeneration (reticulocytes, erythroblasts), often neutrophilic leukocytosis (increased leukopoiesis), and an increase in blood viscosity.

Polycythemia often develops as compensatory process on the basis of 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 concomitant diseases that can explain it. Such cases, by analogy with leukemia, are called erythremia.

3. leukemic syndrome- leukemia (leukemia) or leukemia - develops on the basis of hyperplastic proliferation of leukoblastic tissues hematopoietic system. Clinically, it is manifested mainly by an increase in 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 that are 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 any noticeable, acute leukemia. Much more often, the process proceeds for a long time and in such cases gives a typical picture. chronic leukemia. Finally, there are cases of hyperplasia of one or another leukoblastic tissue without corresponding changes in the white blood, the so-called pseudoleukemias or aleukemic leukemias (myelosis, lymphadenosis and reticuloendotheliosis).

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

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

Chronic leukemic reticuloendotheliosis rare disease- clinically also gives an increase in 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 a large number 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, beriberi) and leading to increased fragility and patency of the capillary walls; the composition and properties of the blood itself at the same time remain without deviations from the norm. These include the so-called purpuras purpura simplex (only skin hemorrhages), purpura rheumatica (accompanied by lesions of the joints), purpura abdominalis (hemorrhage during internal organs abdominal cavity), purpura fulminans (with a very fast fulminant course), as well as scurvy, etc.

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

C) Changes in the chemistry of blood, namely, a slowdown in its coagulability due to a violation (slowdown) of the formation of thrombin (fibrin enzyme) due to a lack of thrombokinase; the rest of the blood is normal, in particular, both the number of platelets and the retractility of the clot, and the duration of bleeding are normal. This belongs

Propaedeutics of childhood diseases: lecture notes by O. V. Osipova

3. Semiotics of damage to the blood system and hematopoietic organs

anemia syndrome. Anemia is understood as a decrease in the amount of hemoglobin (less than 110 g / l) or the number of red blood cells (less than 4 x 1012 g / l). 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. Clinically, anemia manifests itself varying degrees pallor of the skin, mucous membranes. With posthemorrhagic anemia, there are:

1) complaints of patients on dizziness, tinnitus;

2) systolic murmur in the projection of the heart;

3) the noise of the "top" over the vessels.

Children in the first year of life are more likely to have iron deficiency anemia, in children school age- posthemorrhagic, developing after severe or latent bleeding - gastrointestinal, renal, uterine.

To determine the regenerative capacity of the bone marrow, the number of reticulocytes is determined. Their absence in the peripheral blood indicates hypoplastic anemia. The detection of poikilocytes - irregularly shaped erythrocytes, anisocytes - erythrocytes of different sizes is also characteristic. Hemolytic anemia, congenital or acquired, is clinically accompanied by fever, pallor, jaundice, enlargement of the liver and spleen. With acquired forms, the size of erythrocytes is not changed; with hemolytic anemia of Minkowski-Shofar, microspherocytosis is detected.

Hemolysis syndrome is observed in erythrocytopathies, which are based on a decrease in the activity of enzymes in erythrocytes. Hemolytic disease of the newborn is caused by antigenic incompatibility of fetal and maternal erythrocytes either by the Rh factor or by the ABO system, the former being more severe. Red blood cells enter the mother's bloodstream and cause the production of hemolysins, which, as the gestational age increases, pass transplacentally to the fetus and cause hemolysis of red blood cells, which at birth is manifested by anemia, severe jaundice (up to nuclear), enlarged liver and spleen.

In severe cases, fetal death may occur.

Syndromes of leukocytosis and leukopenia are expressed both in an increase in leukocytes (> 10 x 109 / l - leukocytosis), and in their decrease (< 5 х 109/л – лейкопения). Изменение числа лейкоцитов может происходить за счет нейтрофилов или лимфоцитов, реже за счет эозинофилов и моноцитов. Нейтрофильный лейкоцитоз наблюдается при сепсисе, гнойно-воспалительных заболеваниях, причем характерен и сдвиг лейкоцитарной формулы влево до палочкоядерных и юных форм, реже – миелоцитов. При лейкозах может наблюдаться особо высокий лейкоцитоз, characteristic feature which is the presence in the peripheral blood of immature shaped elements (lympho- and myeloblasts). In chronic leukemia, leukocytosis is especially high (several hundred thousand), all transitional forms of leukocytes are determined in the white blood formula. For acute leukemia, hiatus leicemicus is characteristic in the blood count, when both particularly immature cells and a small number of mature cells (segmented neutrophils) without transitional forms are present in the peripheral blood.

Lymphocytic leukocytosis is observed with asymptomatic infectious lymphocytosis (sometimes higher than 100 x 109 / l), whooping cough (20 x 109 / l), infectious mononucleosis. Lymphocytosis due to immature cells (lymphoblasts) is detected in lymphoid leukemia, relative lymphocytosis - in viral infections (influenza, SARS, rubella). Eosinophilic leukemoid reactions (an increase in eosinophils in the peripheral blood) are found in allergic diseases (bronchial asthma, serum sickness), helminthic invasion(ascariasis), protozoal infections (giardiasis). At measles rubella, malaria, leishmaniasis, diphtheria, mumps relative monocytosis. Leukopenias develop more often due to a decrease in neutrophils - neutropenia, which is defined in children as a decrease in the absolute number of leukocytes (neutrophils) by 30% below the age norm, they are congenital and acquired, can occur after taking medications, especially cytostatics - 6-mercaptopurine, cyclophosphamide , as well as sulfonamides, during the period of recovery from typhoid fever, with brucellosis, during a rash with bark and rubella, with malaria. Leukopenia is also characterized by viral infections. Neutropenia in combination with severe anemia is observed in hypoplastic anemia, relative and absolute lymphopenia in immunodeficiency states.

Hemorrhagic syndrome involves increased bleeding: bleeding from the mucous membranes of the nose, hemorrhages in the skin and joints, gastrointestinal bleeding.

Types of bleeding

1. Hematoma type characteristic of hemophilia A, B (deficiency of VIII, IX factors). Clinically, extensive hemorrhages are detected in the subcutaneous tissue, under the aponeuroses, in the serous membranes, muscles, joints with the development of deforming arthrosis, contractures, pathological fractures, profuse post-traumatic and spontaneous bleeding. Develop a few hours after injury (late bleeding).

2. Petechial-spotted, or microcirculatory, type is observed with thrombocytopenia, thrombocytopathies, with hypo- and dysfibrinogenemia, deficiency of X, V, II factors. It is clinically characterized by petechiae, ecchymosis on the skin and mucous membranes, spontaneous bleeding or bleeding that occurs with the slightest injury: nasal, gingival, uterine, renal. Hematomas are rare, there are no changes in the musculoskeletal system, there are no postoperative bleeding, except after tonsillectomy. dangerous frequent hemorrhages in the brain, preceded by petechial hemorrhages.

3. Mixed (microcirculatory-hematoma type) is noted in von Willebrand disease and von Willebrand-Jurgens syndrome, since a deficiency in the coagulation activity of plasma factors (VIII, IX, VIII + V, XIII) can be combined with platelet dysfunction. Of the acquired forms, it can be caused by intravascular coagulation syndrome, an overdose of anticoagulants. It is clinically characterized by a combination of the two indicated above with a predominance of the microcirculatory type. Hemorrhages in the joints are rare.

4. Vasculitis-purple type is the result of exudative-inflammatory changes in microvessels against the background of immunoallergic and infectious-toxic disorders. The most common among this group of diseases is hemorrhagic vasculitis (Schonlein-Genoch syndrome), in which the hemorrhagic syndrome is presented symmetrically located (mainly on the limbs in the area large joints) elements clearly delimited from healthy skin, protruding above its surface, represented by papules, blisters, vesicles, which may be accompanied by necrosis and crusting. Perhaps an undulating course, "blooming" of elements from crimson to yellow color followed by a slight peeling of the skin. In the vasculitic-purple type, abdominal crises are possible with profuse bleeding, vomiting, macro- and microhematuria.

5. Angiomatous type is typical for various forms telangiectasias, most often - Rendu-Osler disease. Clinically, there are no spontaneous and post-traumatic hemorrhages, but there are repeated bleeding from areas of angiomatous altered vessels - nasal, intestinal bleeding, less often hematuria and pulmonary hemorrhage.

Syndrome of enlarged lymph nodes

Lymph nodes can increase in various processes.

1. Acute regional enlargement of lymph nodes in the form local reaction skin over them (hyperemia, edema), soreness is typical for staphylo- and streptococcal infection(pyoderma, furuncle, tonsillitis, otitis media, infected wounds, eczema, gingivitis, stomatitis). If the lymph nodes suppurate, then the temperature rises. A diffuse increase in the occipital, posterior cervical, tonsillar nodes is noted with rubella, scarlet fever, infectious mononucleosis, and acute respiratory viral diseases.

In older children, the submandibular and lymph nodes are especially enlarged with lacunar angina, diphtheria pharynx.

2. When acute inflammation lymphadenitis tends to disappear quickly, persists for a long time with chronic infections (tuberculosis is often limited to the cervical group). The peripheral lymph nodes involved in the tuberculous process are dense, painless, tend to caseous decay and the formation of fistulas, after which they remain irregular shape scarring. The nodes are soldered to each other, with the skin and subcutaneous tissue. With disseminated tuberculosis and chronic tuberculosis intoxication, a generalized enlargement of the lymph nodes with the development of fibrous tissue in the affected lymph nodes can be observed. Diffuse enlargement of mildly painful lymph nodes up to size hazelnut observed in brucellosis. At the same time, these patients have an enlarged spleen. Of the protozoal diseases, lymphadenopathy is observed with toxoplasmosis (an increase in the cervical lymph nodes). Generalized enlargement of the lymph nodes can be observed in fungal diseases.

3. Lymph nodes also increase in some viral infections. The occipital and behind the ear lymph nodes increase in the prodrome of rubella, later noted diffuse magnification lymph nodes, with their palpation there is an elastic consistency, soreness. Peripheral lymph nodes may be moderately enlarged in measles, influenza, adenovirus infection, they have a dense texture and are painful on palpation. With infectious mononucleosis (Filatov's disease), the enlargement of the lymph nodes is significant in the neck on both sides, packets of lymph nodes may form in other areas. An increase in regional lymph nodes with symptoms of periadenitis (solidity with the skin) is found in cat scratch disease, which is accompanied by chills, moderate leukocytosis, suppuration is rare.

4. Lymph nodes can increase in infectious and allergic diseases. Allergic subsepsis of Wissler-Fanconi is manifested by diffuse micropolyadenia.

At the injection site of the serum foreign protein, regional enlargement of the lymph nodes may occur, and diffuse lymphadenopathy is also possible.

5. A significant increase in lymph nodes is observed in blood diseases. As a rule, in acute leukemia, there is a diffuse enlargement of the lymph nodes. It appears early and is most pronounced in the neck. Its size does not exceed the size of a hazelnut, but with tumor forms it can be significant (the lymph nodes of the neck, mediastinum, and other areas increase, they form large packets). Chronic leukemia - myelosis - is rare in children, the enlargement of the lymph nodes is not pronounced.

6. With a tumor process, the lymph nodes often increase, they can become the center of primary tumors or metastases in them. With lymphosarcoma, enlarged lymph nodes are palpated in the form of large or small tumor masses, which then grow into the surrounding tissues, lose mobility, and can compress the surrounding tissues (edema, thrombosis, paralysis occur). An increase in peripheral lymph nodes is the main symptom in lymphogranulomatosis: the cervical and subclavian lymph nodes increase, which are a conglomerate, a package with vaguely defined nodes. They are initially mobile, not soldered to each other and the surrounding tissues. Later, they can be interconnected and the underlying tissues, become dense, sometimes moderately painful. Berezovsky-Sternberg cells are found in the punctate. Enlarged lymph nodes can be found in multiple myeloma, reticulosarcoma.

7. Reticulohistiocytosis "X" is accompanied by an increase in peripheral lymph nodes. Children's "lymphatism" - a manifestation of the peculiarities of the constitution - a purely physiological, absolutely symmetrical enlargement of the lymph nodes that accompanies the growth of the child. At the age of 6-10 years, the total lymphoid mass child's body can be twice the lymphoid mass of an adult, in the future, its involution occurs. Among the manifestations border state health can be attributed to hyperplasia thymus or peripheral lymph glands. Significant thymic hyperplasia requires exclusion tumor process, immunodeficiency states. Significant thymic hyperplasia may develop in children with markedly accelerated physical development, overfeeding with protein. Such "acceleration" lymphatism is noted in children at the end of the first, second year, rarely at 3-5 years.

An anomaly of the constitution should be considered lymphatic-hypoplastic diathesis, in which an increase in the thymus gland and in small degree hyperplasia of peripheral lymph nodes is combined with small indicators of length and body weight at birth and subsequent lag in growth rate and body weight gain. This condition is a consequence of intrauterine infection or malnutrition, neurohormonal dysfunction. In cases where such dysfunction leads to decreased adrenal reserves or glucocorticoid function, the child may have thymic hyperplasia.

Both types of lymphatism - both macrosomatic and hypoplastic - have increased risk malignant course of intercurrent, more often respiratory infections. Against the background of hyperplasia of the thymus, there is a risk of sudden death.

Lymphaticism syndrome, clinically reminiscent of childhood lymphatism, but with a greater degree of hyperplasia of the lymphatic formations and with general disturbances (such as crying, anxiety, body temperature instability, runny nose), develops with respiratory or food sensitization.

In the latter case, due to the increase in mesenteric nodes, a picture of regular colic with bloating occurs, then the tonsils and adenoids increase.

The diagnosis of constitutional lymphatism requires the mandatory exclusion of other causes of lymphoid hyperplasia.

The syndrome of insufficiency of bone marrow hematopoiesis, or myelophthisis, can develop acutely when damaged by penetrating radiation, individual high sensitivity to antibiotics, sulfonamides, cytostatics, anti-inflammatory or painkillers. It is possible to defeat all the sprouts of the bone marrow hematopoiesis. Clinical manifestations: high fever, intoxication, hemorrhagic rashes 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 punctate - depletion of cellular forms of all germs, a picture of cellular decay. More often, hematopoiesis insufficiency in children occurs as a slowly progressive disease.

Constitutional aplastic anemia (or Fanconi anemia) is more often detected after 2-3 years, debuts with monocytopenia, anemia or leukopenia, thrombocytopenia. It is clinically manifested by general weakness, pallor, shortness of breath, pain in the heart, persistent infections, lesions of the oral mucosa, and increased bleeding. Bone marrow failure is accompanied by multiple skeletal anomalies, especially typically aplasia of the radius on one of the forearms. The size of circulating erythrocytes is increased. Acquired insufficiency of hematopoiesis is observed with malnutrition, with high speed loss of blood cells or their destruction. Low efficiency erythropoiesis can occur when there is a lack of erythropoiesis stimulants (renal hypoplasia, chronic kidney failure, thyroid insufficiency.

Alimentary-deficient, or nutritional, anemia develops with protein-energy insufficiency, with an imbalance in the provision of children early age complex of essential nutrients, especially iron. At premature birth children lack the depot of fatty energy substances necessary for the newborn, in particular Fe, Cu, vitamin B12. Hemoglobinopathies in children in Africa, Asia, the Middle East are due to the carriage and genetic heritability of abnormal hemoglobin structures (sickle cell anemia, thalassemia). General manifestations hemoglobinopathies - chronic anemia, spleno- and hepatomegaly, hemolytic crises, multiple organ damage as a result of hemosiderosis. Acute leukemia is the most common form malignant neoplasms in children, they arise mainly from the lymphoid tissue, more often at the age of 2–4 years.

Clinically, there are signs of displacement of normal hematopoiesis with anemia, thrombocytopenia, hemorrhagic manifestations, enlargement of the liver, spleen, and lymph nodes.

The key point in the diagnosis is the statement of the growth of anaplastic hematopoietic cells in the myelogram or bone biopsy.

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DISEASES OF THE HEATING SYSTEM

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Lymphoid system (hematopoietic and immune system organs)

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Lymphoid system (organs of hematopoiesis and immune system) One of the main conditions for human existence is the body's ability to resist the introduction of infection or other foreign substances. This function is performed by the protective system. Immunity (from lat.

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Diseases of the cardiovascular system and hematopoietic organs The vascular system is a powerful branched tree that has roots, trunk, branches, and leaves. Every cell in our body owes its life to a blood vessel - a capillary. Take everything from the body

1. ANEMIA SYNDROME (GENERAL ANEMIC).

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

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

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

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

Inspection. Paleness of the skin and mucous membranes. Dyspnea. Palpation, pulse of weak filling, quickened, filiform. Decreased blood pressure.

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

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

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

2. SYNDROME OF TISSUE IRON DEFICIENCY.

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

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

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

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

Inspection: Smoothness of the papillae of the tongue. Dryness of mucous membranes. Dryness, brittle hair. Striation, brittleness and change in the shape of the nails. Cracks in the corners of the mouth.

Palpation: Dry skin, peeling.

Percussion: Expansion of relative cardiac dullness to the left.

Auscultation: Heart sounds are muffled, quickened.

Laboratory data: In the blood: Decrease in the level of serum iron, increase in the total iron-binding capacity of serum.

In the general blood test: hypochromic anemia, microcytosis, anisocytosis, poikilocytosis.

Instrumental research.

Esophagogastrofibroscopy: atrophic gastritis.

Examination of gastric juice: decrease in gastric secretion (basal and stimulated).

3. HEMOLYSIS SYNDROME.

Definition: Symptom complex due to increased breakdown of red blood cells.

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

Mechanism:

a) increased breakdown of erythrocytes in spleen cells - an increase in the formation of indirect bilirubin,

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

Complaints: Darkening of urine (permanent or paroxysmal), pain in the left hypochondrium, possible chills, vomiting, fever, intense color of feces.

Examination: Icteric staining of the skin and mucous membranes.

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

Laboratory data:

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

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

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

4. HEMORRHAGIC SYNDROME.

Definition: Symptom complex, which is based on increased bleeding.

Causes: Thrombocytopenic purpura (immune origin, or symptomatic thrombocytopenia with inhibition of bone marrow cell proliferation (aplastic anemia), with bone marrow replacement with tumor tissue (hemoblastosis, tumor metastases in the bone marrow), with increased platelet consumption (DIC), with a lack of vitamin B 12 or folic acid); thrombopytopathy (often a hereditary dysfunction of platelets); hemophilia (hereditary deficiency of 8, or 9, or 11 plasma coagulation factors), acquired coagulopathy (deficiency of plasma coagulation factors in many infections, severe enteropathy, liver damage, malignant neoplasms); hemorrhagic vasculitis (immunoinflammatory vascular disease); hereditary violation of the vascular wall of a separate localization (Randu-Osler telangiectasia), hemangiomas (vascular tumors).

Mechanism:

I. Reducing the number of platelets or their functional inferiority;

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

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

These 3 mechanisms correspond to 3 variants of hemorrhagic

syndrome (see below):

	Thrombocytopenia and thrombocytopathy		coagulopathy			Vasopathy
	Gingival, nasal, abdominal and uterine bleeding. Hemorrhages into the skin when rubbing the skin with a hand, measuring blood pressure.		Profuse, spontaneous, post-traumatic and postoperative bleeding. Massive painful hemorrhages in the joints, muscles, fiber.			Spontaneous hemorrhagic rashes on the skin, often symmetrical. Possibly hematuria. Or persistent bleeding of 1-2 localizations (gastrointestinal, nasal, pulmonary)
Inspection and palpation	Painless, non-tense superficial hemorrhages in the skin and mucous membranes, bruises, petechiae.		The affected joint is deformed, painful on palpation. Contractures, muscle atrophy. Hematomas.			Eruptions on the skin in the form of small indurations, symmetrical, then acquire a purple appearance due to soaking with blood. After the disappearance of hemorrhage, brown pigmentation persists for a long time.
Laboratory data
Bleeding time	lengthened
Clotting time			lengthened
Symptom of "twist", "pinch"	Positive		Negative			fickle
Quantity platelets
Retraction of a blood clot	Weak or missing

Thromboplast ogram		Hypocoagulation		Hypocoagulation
Activirova (standardisi forged) partial plate				enlarged
prothrombin				Possible reduction
Activate another time recalcification		enlarged		enlarged
General blood analysis		Possible normochromic (acute posthemorrhagic), or hypochromic (chronic iron deficiency anemia)		Possible normochromic (acute posthemorrhagic) or tapochromic (chronic iron deficiency) anemia	Normochromic (acute posthemorrhagic) or hypochromic (chronic iron deficiency) anemia is possible. Possible leukocytosis, increased ESR.
Urinalysis: hematuria		Possible		Possible	Possible