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APLASTIC (HYPOPLASTIC) ANEMIA

Previously, this anemia was called panmielophthysis - the consumption of bone marrow.

This is a disease of the blood system, characterized by the inhibition of the hematopoietic function of the bone marrow and manifested by the insufficient formation of red blood cells, leukocytes and platelets - pancytopenia. There are congenital (constitutional) and acquired hypoplasias. In 1888

Ehrlich first described the disease in a young woman, in which acute bleeding, fever, deep anemia and leukopenia developed acutely, and at the autopsy there were no signs of blood formation in the bone marrow.

Hematopoietic hypoplasia may be due to a number of factors: myelotoxic effect is exerted by ionizing radiation, chemicals, in particular benzene and its derivatives, antimetabolites, antitumor drugs. Myelotoxic effect develops when antibiotics, especially chloramphenicol, antimalarial drugs.

Less commonly, hypoplastic anemia occurs in tuberculosis, in pregnancy, in liver damage. Sometimes the cause of anemia is difficult to identify - idiopathic form. Exogenous anemia, as mentioned above, is called myelotoxic.

There may be endogenous hypoplastic anemia — this is Fanconi’s family aplastic anemia, probably due to recessive inheritance of a gene from one of the parents. It is not excluded that the development of hypoplastic anemia is associated with damage to stem cells.

At the same time, there is no shortage of blood formation factors; on the contrary, their

the content increased due to incomplete use of reduced bone marrow.

The disease can occur acutely, subacutely and chronically.
In acute cases, the process begins with hemorrhagic syndrome. Often there is a gradual increase in symptoms. Appear weakness, weakness, dizziness, fatigue. Marked waxy

pallor of the skin without yellowness. With the development of thrombocytopenia, hemorrhages occur in the skin, mucous membranes, and in the fundus. Myocardial dystrophy takes place. Often, inflammatory and purulent-necrotic processes join.

Anemia is pronounced, sometimes hemoglobin is reduced to 20-30 g / l, anemia is normal, and the number of reticulocytes is reduced. Leukopenia can reach 1x10.9 / l or less. It is caused mainly by neutropenia. There is a sharp decrease in red blood cells and platelets.

The bone marrow punctate is scanty, the nuclear elements are represented, as a rule, by lymphocytes, few granulocytes and normocytes.

Megakaryocytes are often absent. Trephine biopsy of the Ilium reveals a sharp predominance of fatty bone marrow over the blood, sometimes the absence of the latter.

Fanconi anemia in children (constitutional aplastic anemia) or congenital pancytopenia was described in 1927 in 3 children in the same family. Boys are sick more often. Moreover, the combination of bone marrow hypoplasia with other defects: microcephaly, microphthalmia, delayed sexual development. Inheritance of the disease is autosomal recessive. Probably, there is a defect in stem cells.
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APLASTIC (HYPOPLASTIC) ANEMIA

  1. Aplastic and hypoplastic anemia
    Anemia is called aplastic, when cells in the bone marrow are few or absent, hypoplastic - when the number of these cells is only reduced. Aplasia that extends to erythrocyte progenitors (pure erythrocyte aplasia) may be congenital [Daymonde-Blackfan syndrome (LK Diamond, KDBlackfan), or erythrogenesis imperfecta] or acquired, arising as
  2. Anemia due to bone marrow drainage, hypothyroidism, and aplastic anemia. MYELOPLASTIC SYNDROME
    Despite the large number of studies devoted to the study of hypo-and aplastic anemias, it is still not possible to create a rational classification of these conditions, since not only the issues of pathomorphogenesis, but even the definition of the very concept of "hypo (a) plastic anemia" is debatable. In accordance with modern concepts of the genesis of hypo- and
  3. APLASTIC (HYPOPLASTIC) ANEMIA
    - A disease in which there is a depression of bone marrow hematopoiesis, accompanied by a decrease in the number of red blood cells, leukocytes and platelets. Main clinical manifestations. In aplastic anemia, patients most often complain of weakness, fatigue, bleeding gums, nasal bleeding, unformed patients, and patients who have had their onions. When viewed
  4. ACQUIRED HYPO-APLASTIC ANEMIA
    In contrast to “genuinic” aplastic anemia (Ehrlich type), acquired hypoaplastic anemias, especially acute forms, are caused by certain etiological factors. Of the antibiotics, hypo-aplastic anemias most often cause chlormycetin (chloramphenicol, levomycetin). According to the literature, chlormicetin contains a free nitro group, which has a toxic effect. In a review paper
  5. Hypo and aplastic anemia
    Hypo- and aplastic anemias are a heterogeneous group of diseases, characterized by a sharp inhibition of blood formation. Differences between hypo-and aplastic anemias are mainly due to the degree of inhibition of bone marrow hematopoiesis. Naturally, this distinction leaves its mark on the clinical manifestations of the disease, the effectiveness of the treatment applied and
  6. Aplastic anemia
    Aplastic anemia (AA) - team is a group of diseases whose main symptom is depression of bone marrow hematopoiesis according to aspirate and biopsy of bone marrow and peripheral pancytopenia (anemia of varying severity, thrombocytopenia, and leykogranulotsitopeniya reticulocytopenia) in the absence of diagnostic signs of leukemia, myelodysplastic syndrome,
  7. CONGENITAL HYPO & APPLASTIC ANEMIA
    Familial aplastic anemia (Fanconi type) For the first time this peculiar anemia was described under the name of children's familial aplastic myelosis. The disease usually develops in childhood, between 4 and 10 years. Known rare cases of development of Fanconi type anemia in adults; in particular, Rohr described a case of marital illness in two brothers aged 20 and 24 years. Anemia carries
  8. Congenital aplastic anemia
    Constitutional aplastic anemia (Fanconi anemia) It proceeds with the suppression of all sprouts of hemopoiesis and congenital anomalies of development. At least 900 cases of Fanconi anemia have been described. It is inherited in an autosomal recessive manner, there are familial forms of the disease in brothers and sisters. It has been established that the group of patients with Fanconi anemia is heterogeneous genetically - at least
  9. Acquired aplastic anemia
    Clinic acquired AA differs depending on the total or selective lesion of hemopoiesis. In patients with acquired AA, unlike hereditary forms, there are no congenital anomalies of development, the physical and mental development of children is not changed, the bone age corresponds to the passport age. For total forms of AA, a combination of hemorrhagic, anemic and
  10. Hypo-and aplastic anemia. Syndrome of bone marrow failure
    Anemia of this group can be acquired (secondary) and hereditary, congenital (primary). Acquired forms may develop under the influence of physical (ionizing radiation); chemical (benzene, arsenic, etc.) factors, drugs (some antibiotics, sulfonamides, antimetabolites - methotrexate, etc.), as well as due to a lack of hormones (myxedema,
  11. Lymphatic-hypoplastic diathesis
    Lymphatic-hypoplastic diathesis is a set of hereditary congenital and acquired morphological and functional features of a child's body, which determine the possibility of hypertrophy of the thymus gland and lymphatic apparatus in children, adrenal hypoplasia, thyroid and parathyroid glands, elements of the cardiovascular system and some other
  12. Hypoplastic anemia (code D 61)
    Definition A pathological condition in which pancytopenia is caused by a decrease in bone marrow hematopoiesis. Etiology, pathogenesis. Etiological factors: • autosomal and recessively inherited genetic defect of stem cells; • ionizing radiation; • drug intoxication; • idiopathic forms (most common). Three are considered possible in pathogenesis.
  13. Classification of aplastic anemia
    Depending on whether there is an isolated inhibition of erythroid germ or all germs, there are partial and total forms of aplastic anemia. They are accompanied by appropriately isolated anemia or pancytopenia. According to the classification of V. I. Kalinicheva (1983), the following variants of the disease are distinguished. I. Hereditary aplastic anemia. 1. Hereditary aplastic
  14. Hypoplastic (aplastic) anemia
    The essence of hypoplastic (and aplastic) anemia consists in a sharp inhibition of bone marrow hematopoiesis, which is accompanied by a decrease in the number of erythrocytes, leukocytes and platelets. Also known is the partial form of hypoplastic anemia with inhibition of the formation of only red blood cells. 479 Aplastic anemia (AA) is a relatively rare disease. It meets with the frequency
  15. Lymphatic-hypoplastic diathesis
    -hyperplastic sm: generalized hyperplasia of lymphoid tissue -sm functional changes in the nervous system: depression, lethargy, weakness, decrease in tendon reflexes -R: increase in mediastinal lymph nodes, thymomegaly -hypoplastic sm-m: hypoplasia of the genital organs, dysfunction and hypomeglia; , drip heart, functional lability of the cardiovascular system
  16. Lympatico-Hypoplastic Diathesis
    Lymphatic-hypoplastic diathesis (LGD) is a congenital dysregulatory endocrinopathy, manifested by impaired adaptation to the external environment and transient immunological insufficiency; a condition characterized by hyperplasia of the lymphoid tissue, transient immunodeficiency, insufficient development of chromaffin tissue. E p and d e m and about l about g and I. Detected more often in children from 2 to 6 years with a frequency of about
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