The main diseases of the blood in children

In children, especially the early age, the most common is a blood disorder, like anemia. Anemia is defined as a decrease in the amount of hemoglobin (less than 110 g / l), or the number of red blood cells (less than 4x10 ¹² / l), or both. Depending on the degree of decrease in the hemoglobin content, the lungs (Hb 90-110 g / l), medium-heavy (Hb 60-80 g / l) and heavy (Hb less than 60 g / l) form of anemia are distinguished.

Anemia in children is clinically manifested by varying degrees of pallor of the skin and visible mucous membranes. In acute anemia (posthemorrhagic), patients complain of dizziness, noise in the ears, a systolic murmur is heard above the heart, and the "top" noise on the vessels. Most often in children of the first three years, iron deficiency anemia is noted, in children of school age - posthemorrhagic, developing after pronounced or hidden bleeding (especially gastrointestinal, renal and uterine). In patients with anemia, it is important to know the regenerative capacity of the bone marrow. For this, the number of reticulocytes is determined. Reticulocytosis always indicates a sufficient regenerative function of the bone marrow. At the same time, the absence of reticulocytes in the peripheral blood or very low values (not corresponding to the degree of anemia) may be one of the signs of hypoplasia (hypoplastic anemia).

In anemia, as a rule, erythrocytes of irregular shape (poikilocytosis) and different sizes (anisocytosis) are found. A special place is occupied by hemolytic anemia. They can be congenital or acquired. Clinically, hemolysis is often accompanied by an increase in body temperature, pallor and varying degrees of jaundice, an increase in the liver and spleen. With hemolytic anemia Minkowski-Schoffar observed microspherocytosis. With the acquired hemolytic anemia, the size of the erythrocytes is usually unchanged.

Often, hemolysis syndrome is observed in erythrocytopathies, which is based on a decrease in the activity of enzymes in erythrocytes, with hemoglobinopathies, in which there is an inborn violation of the structure of the globin part of hemoglobin.

A special place is occupied by hemolytic disease of newborns, caused by the antigenic incompatibility of the erythrocytes of the fetus and the mother. This incompatibility can be in the Rh factor (LI) or in the ABO system. The first form is more severe. In these cases, the red blood cells of the fetus penetrate the mother's blood and cause the production of hemolysins. The maternal hemolysins pass to the fetus transplacentally as the gestational age increases and cause hemolysis in it, which clinically at birth is manifested by anemia, severe jaundice (up to the nuclear one), and enlargement of the liver and spleen. In particularly severe forms, the fetus may die (dropsy of the fetus).

Leukocytosis and leukopenia in children

Changes in white blood can be expressed in an increase and decrease in the number of leukocytes. The increase in the number of leukocytes (in children above 10x10 ⁹ / l) is called leukocytosis, a decrease (less than 5x10 ⁹ / L) _ leukopenia. It is important to know, due to what form elements of white blood there is an increase or decrease in the number of leukocytes. The change in the number of leukocytes can often occur due to neutrophils or lymphocytes. Less frequently observed changes in the number of eosinophils and monocytes. Neutrophilic leukocytosis - absolute neutrophilia - is characteristic of septic and purulent-inflammatory diseases (sepsis, pneumonia, purulent meningitis, osteomyelitis, appendicitis, purulent cholecystitis). Neutrophilia with purulent-septic diseases is accompanied by some rejuvenation - a shift in the leukocyte formula to the left until the stab and young, less often to the myelocytes. Less pronounced neutrophilia in diphtheria, scarlet fever. In malignant diseases of the blood in children - hemopathies (especially in leukemia) - especially high leukocytosis can be observed, the characteristic feature of which is the presence in the peripheral blood of immature uniform elements (lympho- and myeloblasts). With chronic leukemia, leukocytosis is particularly high (several hundred thousand), and in the white blood formula there are all transitional forms of leukocytes. In acute leukemia, the blood formula usually has a hiatus leicemicus, when in the peripheral blood there are both especially immature cells, and in a small number of mature (segmented neutrophils) without transitional forms. Lymphocytic leukocytosis - absolute lymphocytosis - is characteristic of asymptomatic infectious lymphocytosis (sometimes higher than 100x10 ⁹ / L), whooping cough - (20 ... 30) x 10 ⁹ / l, infectious mononucleosis. With the first two diseases - lymphocytes are mature, with the same contagious mononucleosis of unusual form - wide-cytoplasmic. Lymphocytosis due to immature cells - lymphoblasts - is characteristic of lymphoid leukemia. Relative lymphocytosis is noted in viral infections (influenza, acute respiratory-viral diseases, measles, rubella, etc.).

Eosinophilic leukemoid reactions in the form of an increase in the number of eosinophils in peripheral blood are characteristic of allergic diseases (bronchial asthma, serum sickness), helminthiasis (ascaridosis, toxocarosis, etc.) and protozoal infections (giardiasis, etc.). Sometimes monocytic leukemoid reactions are observed, the nature of which is not always clear. Relative monocytosis is characteristic of measles rubella, malaria, leishmaniasis, diphtheria, Vincent-Simanov angina, epidemic parotitis, etc.

Leukopenia is more often observed due to a decrease in the neutrophil count - neutropenia. Neutropenia in children is considered to decrease the absolute number of leukocytes (neutrophils) by 30% below the age norm. Neutropenia can be congenital and acquired. Often they occur after taking medications (especially cytostatic drugs - 6-mercaptopurine, cyclophosphamide, etc., used in the treatment of cancer patients, as well as sulfonamides, amidopyrin), during recovery from typhoid fever, with brucellosis, rash in measles and rubella , with malaria. Leukopenia is characteristic of viral infections, as well as a number of diseases characterized by a particularly severe course.

Neutropenia in combination with severe anemia is noted with hypoplastic anemia. Relative and absolute lymphopenia is observed in immunodeficient conditions. It develops a few months after the onset of clinical signs of immunodeficiency (mainly due to T-lymphocytes).

Hemorrhagic syndrome in children

The term "hemorrhagic syndrome" means increased bleeding in the form of bleeding from the mucous membranes of the nose, the appearance of hemorrhages in the skin and joints, gastrointestinal bleeding, etc. In clinical practice, it is advisable to distinguish several types of bleeding.

1. In the hematoma type, extensive hemorrhages in the subcutaneous tissue, under aponeuroses, in serous membranes, in muscles and joints with the development of deforming arthrosis, contractures, pathological fractures are determined. There are profuse post-traumatic and post-operative hemorrhages, less often spontaneous bleeding. The late character of bleeding is expressed, i.e., a few hours after the trauma. The hematomic type is characteristic for haemophilia A and B (deficiency of VIII and IX factors).
2. Petechial-spotted, or microcirculatory, type is characterized by petechiae, ecchymosis on the skin and mucous membranes, spontaneous or arising at the slightest trauma by bleeding - nasal, gingival, uterine, renal. Hematomas are rare, the musculoskeletal system does not suffer. Postoperative bleeding, except for bleeding after tonsillectomy, is not observed. Frequent and dangerous hemorrhages in the brain; as a rule, they are preceded by petechial hemorrhages in the skin and mucous membranes. Microcirculatory type is observed with thrombocytopenia and thrombocytopathy, hypo- and dysfibrinogenemia, deficiency of X, V and II factors.
3. Mixed (microcirculatory-hematoma) type is characterized by a combination of the two previously listed forms and some features: the microcirculatory type prevails, the hematoma type is negligible (hemorrhage predominantly in the subcutaneous tissue). Hemorrhages in the joints are rare. This type of bleeding is observed in von Willebrand's disease and Willebrand-Jurgens syndrome, as the deficiency of the coagulant activity of plasma factors (VIII, IX, VIII + V, VII, XIII) is combined with platelet dysfunction. From the acquired forms, this type of bleeding can be caused by the syndrome of intravascular coagulation, overdose of anticoagulants.
4. The vasculitic-purple type is caused by exudative-inflammatory phenomena in microvessels against the background of immunoallergic and infectious-toxic disorders. The most common disease of this group is hemorrhagic vasculitis (or Shenlaine-Henoch syndrome). The hemorrhagic syndrome is represented symmetrically located, mainly on the extremities in the region of large joints, with elements clearly delimited from healthy skin. Elements of the rash protrude above its surface, are represented by papules, blisters, blisters, which can be accompanied by necrosis and crusting. Perhaps an undulating flow, the "flowering" of the elements from purple to yellow, followed by a fine peeling of the skin. In the vasculitic-purple type, abdominal crises are possible with heavy bleeding, vomiting, macro- and (more often) microhematuria.
5. Angiomatous type is characteristic for various forms of telangiectasias. The most frequent type is Rundu-Osler's disease. With this type of bleeding, there are no spontaneous and posttraumatic hemorrhages in the skin, subcutaneous tissue and other organs, but there are repeated bleeding from the sites of angiomatologically-altered vessels - nasal, intestinal, less often - hematuria and pulmonary.

Clinical isolation of these variants of bleeding allows to determine a set of laboratory studies necessary to clarify the diagnosis or cause of hemorrhagic syndrome.

Insufficiency of bone marrow hematopoiesis

Myelopthisis can develop rapidly when there is a lesion of some myelotoxic factors, for example, a large dose of benzene or penetrating radiation. Sometimes such a reaction occurs in children due to individual high sensitivity to antibiotics (eg, levomycetin), sulfonamides, cytostatics, anti-inflammatory or analgesic drugs. With total defeat of all germs of bone marrow hematopoiesis speak of "panmyelophthisis", or total aplasia of hemopoiesis. Common clinical manifestations may include high fever, intoxication, hemorrhagic rashes or bleeding, necrotic inflammation and ulcerative processes on the mucous membranes, local or generalized manifestations of infections or fungal infections. In the blood - pancytopenia in the absence of signs of blood regeneration, in the puncture of the bone marrow - impoverishment of the cellular forms of all the shoots, a picture of cell decay and devastation.

Much more often, the insufficiency of hematopoiesis in children manifests itself as a slowly progressing disease, and its symptoms correspond to the most involved germ of the hematopoiesis. In pediatric practice, patients with congenital constitutional forms of hematopoietic insufficiency may be found.

Constitutional aplastic anemia, or Fanconi anemia, occurs in the most typical cases after 2-3 years, but sometimes even in high school age. It debuts the disease from the onset of monocytopenia or anemia, either leukopenia or thrombocytopenia. In the first stage, the cause for treatment is general weakness, pallor, dyspnea, pain in the heart. At the second variant - persistent infections and lesions of the mucous membrane of the oral cavity, with the third version of the debut - increased bleeding and bruising on the skin. Within a few weeks, sometimes months and rarely longer, there is a natural transition to bitsitopenia (two sprouts) and, finally, pancytopenia of peripheral blood. Bone marrow failure in most patients is accompanied by multiple anomalies of the skeleton and is especially typical of aplasia of one of the radial bones. Actually anemia with such pancytopenia differs by a clear tendency to increase the size of circulating erythrocytes (macrocytic anemia), and often leukocytes. A cytogenetic study confirms the effect of increased "fragility" of chromosomes in lymphoid cells.

The most significant congenital diseases, accompanied by a monocytopenic syndrome for peripheral blood, are presented below.

Erythroblastic aplasia:

• congenital hypoplastic anemia;
• Blackfen-Daymond;
• transient erythroblastopenia of childhood;
• transient aplasia in diseases with chronic hemolysis of erythrocytes.

Neutropenia:

• Kostmann's disease;
• Schweckman-Daimond syndrome;
• cyclic neutropenia.

Thrombocytopenia:

• thrombocytopenia during aplasia of the radius;
• amygakaryocytic thrombocytopenia.

Many blood diseases in children, as well as non-hematological diseases, are manifested by cytopenic syndromes regardless of congenital insufficiency of bone marrow hematopoiesis. In these cases, either the transient acquired low hematopoietic productivity is observed, as happens, for example, in malnutrition, or the relative insufficiency of hematopoiesis at a high rate of loss of blood cells or their destruction.

The low efficiency of erythropoiesis, which simulates clinically hypoplastic anemia, can occur when the natural physiological stimulants of erythropoiesis are deficient. These include kidney hypoplasia or chronic renal failure with loss of erythropoietin production.

Insufficiency of the thyroid gland is also often manifested by persistent anemia. Sometimes the cause of the inhibition of erythropoiesis can be seen in several factors of the pathogenesis of the underlying somatic disease, including both eating disorders, and the myelodepressant effect of chronic inflammation, and the frequent side effects of the drugs used.

Alimentary-deficient, or "nutritive," anemia

In countries or regions with a high prevalence of starvation of children in terms of protein-energy deficiency anemization is a natural companion of hunger and genetically always poly-physiological. Along with the factors of food insufficiency, in its origin a serious role is played by numerous acute and chronic infections, helminthiases and parasitosis. In countries with a slightly more organized life and sanitary culture, nutritional anemia is most often detected in young children, where a limited assortment of food does not allow balanced supply of the whole complex of necessary nutrients. Especially critical is the availability of iron for children born prematurely or with low body weight. In preterm birth, the child is deprived of a period of accumulation of nutrients (deposition), which is related to the terms of the last weeks of pregnancy. They lack the fatty energy substances necessary for the newborn, and, in particular, iron, copper and vitamin B12. Breast milk, especially in a malnourished nursing mother, can not compensate for the lack of deposited nutritional components. Deficiency of iron is a danger to oxygen supply both through a deficiency of hemoglobin of the blood, and through violations of tissue mechanisms of oxygen transfer from the blood to tissues. Hence the special alertness of pediatricians observing children of an early age in relation to the provision of the child with adequate nutrition and preventing his anemization. The introduction of whole cow's milk or its mixtures into the baby's nutrition can also affect the provision of iron at the end of the 1-2 year of life. Here often there are losses of iron with erythrocytes, which appear in the lumen of the intestine with diapedesis. Finally, in the adolescent period, especially in girls who started menstruating, there is again a high probability of insecurity with iron and the appearance of anemia. Pediatric practice uses several laboratory approaches to detect iron deficiency, in particular through the determination of ferritin content, iron transferrin saturation, etc. However, the first line of diagnosis is undoubtedly hematological studies aimed at a relatively early detection of initial signs of anemia.

The list of nutrients, the deficiency of which naturally leads to anemia, and sometimes leukopenia, can be quite broad. The combination of iron and copper deficiencies at an early age and with eating disorders in all age groups has already been indicated. Cases of megaloblastic anemia in children with deficiency of vitamin or folic acid or thiamine, hypochromic anemia with vitamin B6 deficiency, hemolytic anemia with a deficiency of vitamin E in low-fat children are described.

[1], [2], [3], [4], [5], [6], [7], [8],

Hemoglobinopathies in children

They are quite widespread among representatives of ethnic groups that have emerged from Africa, Asia, the Middle East and the Mediterranean. Diseases of this group are due to the carriage and genetic inheritance of abnormal globin structures in hemoglobin. Representatives of this group, the most common, are sickle cell anemia and thalassemia (large and small). Common manifestations of hemoglobinopathies are chronic anemia, splenic and hepatomegaly, hemolytic crises, polyorganous lesions due to hemosiderosis or hemochromatosis. Intercurrent infections cause crises in the underlying disease.

The key to recognition is the biochemical study of hemoglobin. Recognition is possible already in the first trimester of pregnancy according to trophoblast biopsy data.

Acute leukemia in children

Leukemia is the most common form of malignant neoplasm in children. At the same time, the overwhelming majority of acute leukemias come from lymphoid tissue (85%). This is probably due to the exceptionally rapid growth rate of lymphoid formations in children, which exceeds the growth rates of any other organs and tissues of the body. In addition to the most powerful growth stimulation through the system of growth hormone and insulin, lymphoid formations are additionally stimulated by numerous infections, immunizations, and traumas. It was revealed that the "peak" of the frequency of childhood leukemia occurs in the age range of 2 to 4 years, and the highest frequency of leukemia is observed in the most well-off families, household environment and nutrition of children. A peculiar exception are children with Down's disease who also have a high risk of leukemia.

In the clinical picture of leukemia, there are signs of displacement of normal hematopoiesis with anemia, thrombocytopenia and often hemorrhagic manifestations, hyperplastic changes in the hematopoietic organs - enlargement of the liver, spleen, lymph nodes, often gums, testicles in boys and any internal organs to which tumor proliferation is spread. The main way in diagnosis is to ascertain the growth of anaplastic hematopoietic cells in a myelogram or bone biopsy specimens. For more than 20 years, acute lymphoblastic leukemia in children has ceased to be a fatal disease. The use of modern schemes of polychemotherapy, sometimes in combination with bone marrow transplants, guarantees either a long-term survival or a practical cure in most patients.

Other morphological forms of acute leukemia can proceed more stubbornly, and the long-term results of their treatment are still somewhat worse.

[9], [10], [11], [12], [13], [14], [15], [16], [17], [18]