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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 aplastic states of blood formation, we distinguish two main groups of hypo-and aplastic anemias: 1) congenital (“genuinic”) hypo-aplastic anemias; 2) acquired hypo-aplastic anemias of various etiologies.

With the flow there are acute, subacute and chronic forms. Hypoaplastic anemia may be complicated by hemolytic, hemorrhagic, or agranulocytic-septic syndrome.

With all the variety of etiological and clinical forms, all hypo- and aplastic states of hematopoiesis are united by a single pathomorphological substrate: these are panmielopathy, in other words, total hematopoietic suppression, impaired proliferation (and to a lesser degree of differentiation) of the most primordial elements of hemopoiesis - hemogist and hemocytoblasts.

A reflection of the state of panmielopathy (paresis, phthiasis) is pancytopenia in the peripheral blood. Any hypo-and aplastic anemia occurs with concomitant leuko- and thrombocytopenia. Speaking of "anemia", we only emphasize the main syndrome - anemic, causing the uniqueness of the clinical picture of the disease.

Etiology. The etiology of hypo-and aplastic anemias is very diverse. In the development of myelophilis play a role as endogenous, still unknown causes, as well as exogenous factors.

It is noteworthy that the aplastic state of blood formation may occur under the influence of such factors as ionizing radiation, benzenes, which in experimental conditions lead to the development of a systemic blood disease, leukemia (see below).



Etiological factors in the development of hypo-and aplastic anemias



I. ENDOGENOUS

1. Endocrine - hypothyroidism, hypopituitarism (Fanconi disease); benign tumors (lymphoepithelioma) of the thymus gland.

2. Generic - Ehrlich aplastic anemia.

3. Osteomyelosclerosis, osteopetrosis (Albers-Schoenberg marble disease).

Ii. Exogenous

1. Physical radiation injuries (X-rays, radium, isotopes, atomic energy).

2. Chemical:

a) toxic - benzene (benzenes);

b) cytostatic: chloroethylamines (embichin, dopan), trichlorethylenemelamines (TEM, TEF, TioTEF), colchicine, carbon tetrachloride, folic acid antagonists (aminopterin), antipurines (6-mercaptopurine, chloropurine), urethane, myrethyroid, myrethyrene, antipurines (6-mercaptoptopurine, chloropurine), urethan, myrethystrophins, antipurines (6-mercaptoptopurine, chloropurine), urethan, myrethystrophins, antipurines (6-mercaptoptopurine, chloropurine), urethan, myrethyne, myrethane, anesthetic .

3. Toxic-allergic:

a) medication: pyramidone, atofan, methylthiouracil, arsenobenzenes (salvarsan), barbiturates, sulfonamides, tibon, PAS, gold salts, anti-epileptic (hydantoin);

b) antibiotic: chlormicetin (chloramphenicol).

4. Infectious (presumably): generalized, with necrotic lesions of the bone marrow, forms of organ tuberculosis, syphilis, brucellosis, typhoid fever, salmonellosis, severe septic diseases.



We do not consider splenogenic pancytopenia associated with functional moments, that is, delayed aging or delayed leaching, to the hypo- and aplastic states of blood formation, since under these conditions the bone marrow is not hypoplastic, but, on the contrary, hyperplastic, there is only a violation of aging and output in the circulation of blood cells, which is proved by the "dramatic" effect of splenectomy.

Some authors refer to hypoplastic conditions and anemia (pancytopenia) associated with the processes of metaplasia in systemic diseases (reticulose - leukemia) or bone marrow metastasis (carcinosis). We suppose that methodically it is more correct to refer these states to systemic, i.e. hyperplastic, diseases, and anemia in these conditions is referred to as metaplastic, since the bone marrow is essentially not aplasian, it is anaplastic or metaplazed, i.e. replaced by atypical leukemia or blastomatous tissue.

This definition does not contradict the well-known cases of the development of true bone marrow aplasia with a picture of panmielophthisis as a terminal state in systemic hyperplastic myeloproliferative and lymphoproliferative processes. Even a purely proliferative disease such as erythremia, which is essentially panmielosis, in the process of natural development and involution, especially after treatment with P32, sometimes ends with a picture of the hypo-aplastic hematopoietic state.

Recognizing the commonality of hypoplastic and aplastic anemias, the presence of transitional stages between them, we at the same time consider it necessary to emphasize the differences, both quantitative and qualitative, that exist between these hematopoietic states.
These differences, in our opinion, have not only theoretical, but also practical significance from the point of view of the final prognosis and the possibilities of effective treatment of patients.

With aplastic anemia, there is a deeper damage to the original elements of the blood, perhaps at the stage of reticular cells (hemogistioblasts), as a result of which the hemopoietic tree dries out in the cortex itself, even before the development of the blood cells themselves. Therefore, in aplastic states, as a rule, there are no signs of impaired maturation of blood cells associated with a deficiency of specific blood formation factors (B12, folic acid, iron). Even if these symptoms exist, it is not as the main factors, but as additional pathogenetic factors for the development of anemia. In other words, in aplastic conditions, there is not so much a qualitative as a quantitative hematopoietic disorder, manifested in the reduction of the hematopoietic springboard, and this reduction is progressive, reaching in severe cases to complete aplasia, i.e., atrophy of the bone marrow as a blood-forming organ.

On the contrary, in hypoplastic conditions (including the hypoplastic stage of aplastic anemia), as a rule, at a certain stage of the evolutionary development of the pathological process, phenomena of a qualitative hematopoietic disorder are noted, mainly due to deficiency of the hematopoietic vitamins - vitamin B12 and folic (folinic) acid. This qualitative deficiency of hemopoiesis is reflected in the appearance of the corresponding dystrophic, forms - “megaloblastoid” erythroblasts, polysegmentome neutrophils, etc.

A similar picture, described by us (1948) as aregenerative dysplastic anemia, according to modern concepts corresponds to the concept of B12-achrestic anemia. At the same time, the content of vitamin B12 in the blood, according to a number of authors, is increased. Therefore, this is not about the insufficient supply of vitamin B12 (folinic acid) to the bone marrow, but about the non-use (ahresia) by the bone marrow of vitamins necessary for normal blood formation.

Due to the qualitative and quantitative differences in the nature of blood formation in hypo-and aplastic anemias, the state of blood exchange is different.

If in typical aplastic anemia, blood metabolism is drastically reduced, as evidenced by a number of tests (pale blood serum, low bilirubin, iron content, no urobilirubin), then in hypoplastic conditions occurring with a less significant reduction of the hemopoiesis springboard, blood decomposition products not used bone marrow, often give a picture imitating hemolytic anemia and jaundice (icteric serum with a high content of bilirubin and iron, urobilinuria, bile pleiochromia, hypers erkobilinofekaliya). This is the so-called hypoplastic anemia with a hemolytic component.

A characteristic feature of this form is the combination of symptoms of increased hemolysis with the hypoplastic state of blood formation, characterized by the suppression of all functions of hemopoiesis. This is the main difference of this anemic form from true hemolytic anemia. Peripheral blood is characterized by pancytopenia with normal or slightly elevated (not an adequate degree of hemolysis!) Reticulocytosis. In some cases, the Coombs test is positive.

These differences between aplastic and hypoplastic anemias are, to a certain extent, arbitrary. There are many transitional forms between hypo- and aplastic states, just as there are many clinical variants of these states. Meanwhile, the question of whether the observed particular patient has aplastic or hypoplastic anemia at this stage is very significant. This question can often be resolved only by dynamic observation, taking into account the effectiveness of therapy.

Directly studying punctates and trepanates of the hematopoietic organs (bone marrow, liver, spleen), as well as radiometric methods using radiol active iron isotopes (Fe59) and chromium - 51, are very essential for determining the extent of surviving blood formation and the possible presence of extramedullary foci of hematopoiesis.

Radioactive chromium (Cr51) is also used to mark red blood cells and determine the average duration of stay (“life”) of red blood cells in circulating blood.
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