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CHAPTER 9. BLOOD AND IMMUNE SYSTEM DISEASES

Diseases of blood and immune system, including anemia, hemorrhagic pathology (coagulopathy, thrombopathy, vasopathy, DIC) are widespread among the pediat-ric population. The incidence of immunodeficiency-associated lymphoproliferative disorders caused by environmental and toxicological factors is gradually increasing. Leukemia accounts for up to 1/3 of all malignancies in children. In most cases, blood disorders in children develop gradually, without vivid clinical manifestations. The doctor must identify non-specific symptoms of hematological disorder in good time, be able to diagnose it and contribute to cure.

ANEMIA

Anemia is a pathological condition characterized by a decrease in the number of red blood cells per unit volume of blood and alterations in hemoglobin concentration. In children, anemia can be an independent disease or arise secondary to various pathological conditions and diseases.

Currently, there is no universal classification of anemias (table 9.1).

Table 9.1. Classification of anemia (Idelson L.I. et al., 1979)

Occurrence mechanism

Type

Form

I. Blood loss anemia

Acute posthemorrhagic anemia

Chronic posthemorrhagic anemia

II. Anemia associated with impaired hemato-poiesis

1. Anemia due to impaired hemoglobin synthesis

Iron-deficiency anemia

Anemia associated with impaired synthesis or utilization of porphyrins

2. Anemia due to impaired DNA and RNA synthesis (megaloblastic anemia)

Vitamin B12 deficiency anemia

Folate-deficiency anemia

3. Anemia associated with impaired red blood cell production (dyserythropoietic anemia)

Inherited. Acquired

4. Anemia associated with total or partial inhibition of hemopoiesis (hypoplastic and aplastic anemia)

Inherited. Acquired

End of the table 9.1

Occurrence mechanism

Type

Form

III. Hemolytic anemia

1. Inherited hemolytic anemia

Anemia associated with red blood cell membrane damage (Min-kowsky-Chauffard syndrome, etc.)

Anemia due to impaired activity of red blood cell enzymes

Anemia associated with impaired structure or synthesis of hemoglobin

2. Acquired hemolytic anemia

Antibody-induced anemia

Anemia associated with structural membrane changes due to somatic mutation (Marchiafava-Micheli syndrome)

Anemia associated with mechanical damage to red blood cells

Anemia associated with chemical damage to red blood cells

Nutritional or vitamin deficiency anemia

Anemia associated with parasite-induced damage of red blood cells (malaria-related)

Description of the anemia pattern should include the degree of disease severity, which depends on the hemoglobin concentration and red blood cell count (table 9.2).

Table 9.2. Degrees of anemia severity

Degree of anemia

Hemoglobin, g/L

Red blood cells, ×1012/L

I - mild

110-90

3.5-3.0

II - moderate

89-70

2.9-2.5

III - severe

<69

<2.5

According to the color index (CI), anemias are subdivided into normo-, hypo-and hyperchromic. This information is essential in assessing the nature of the disease

(table 9.3).

Table 9.3. Type of anemia according to color index

Type of anemia

Color index

Nosological entity

Hypochromic

<0.85

Iron deficiency anemia, chronic posthemorrhagic anemia, sideroblastic anemia, thalassemia

Normochromic

0.85-1.0

Acute posthemorrhagic anemia, hemolytic anemia, hypoplastic anemia, neoplastic anemia, etc.

End of the table 9.3

Type of anemia

Color index

Nosological entity

Hyperchromic

>1,0

Vitamin B12 deficiency anemia, folic acid deficiency anemia, subacute and chronic aplastic anemia, drug-induced anemia, etc.

Anemias can be classified as hypo-, normo-, and hyperregenerative according to the erythropoiesis status (table 9.4); this information is necessary for the evaluation of the state of bone marrow hematopoiesis and prognosis of the disease.

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