HYPERGLOBULIAS (Greek erythros red + kytos a receptacle, here — a cell + - osis; ustar. a polyglobulia) — increase in blood of a hemoglobin content and quantity of erythrocytes.
Distinguish absolute E., at to-rykh the mass of the circulating erythrocytes, and relative, caused by reduction of volume of a blood plasma is increased.
In fiziol. conditions erythrocyte balance in an organism is maintained by interaction of such factors as saturation of blood oxygen, the content of erythropoetin (see) in blood, functional capacity of marrow and mass of the circulating erythrocytes.
For the first time on a possibility of existence compensatory E., developing at a high-rise hypoxia, specified P. Bert in 1878. Further existence E was noted. at a number of diseases.
By origin absolute E. divide on primary (hereditary), caused by genovariations, and secondary, or symptomatic (acquired). Secondary E. meet at various somatopathies as a result of the mediated impact on an erythro-cytopoiesis of erythropoetin.
Absolute E. (both primary, and secondary) divide also on hypoxemic and negipoksemiches-ky. Absolute hypoxemic E., caused by an anoxemia and in response to it physiologically adequate raised products of erythropoetin, can be noted as a result of reduction of transport of oxygen at hron. diseases slight, inborn «blue» heart diseases (see Heart diseases inborn), stay in the mountain area (see. A mountain disease), methemoglobinemias (see), carboxyhaemo-globinemii (see Carbon monoxide), a pikkviksky syndrome (see). They can be observed in case of reduction of release of oxygen at hemoglobinopathies (see) with the increased affinity to oxygen and hereditarily the caused decrease in contents 2,3-diphosphoglyceric to - you in erythrocytes.
Absolute not hypoxemic E., not connected with an anoxemia and caused by physiologically inadequate raised products of erythropoetin, meet at such damages of kidneys as cancer, a polycystosis, a renal artery stenosis (see Kidneys), a hydronephrosis (see), after operation of renal transplantation, and also at the tumors which are followed by existence of the ectopic centers of products of erythropoetin, napr at adenoma of adrenal glands (see Adrenal glands, tumors), a hromaffinoma (see), a fibromyoma (see). Excess products of erythropoetin are noted at a so-called nasledstvennosemeyny hyperglobulia (see the Hyperglobulia hereditary and family).
Relative hyperglobulias are quite often observed at arterial hypertension, at smokers, at the persons abusing alcohol or being in a state hron. stress.
Manifestations E. are various and, as a rule, are defined by features of the basic patol. process in this connection the symptomatology can vary them. Red cyanosis of skin and visible mucous membranes, moderate gepato-and a splenomegaly, a varicosity, and sometimes fibrinferments and bleedings are usually noted. In a gemogramma increase in indicators of red blood comes to light (ShM. Gemogramma) and a hematocrit (see Gematokritny number). Quantity of leukocytes and thrombocytes usually within norm.
AA. establish on the basis of the anamnesis, symptoms of diseases, to-rye can be followed by E., the indicators of blood given to a trepanobiopsiya of an ileal bone (see T a repanobiopsiya).
AA. it is necessary to distinguish from a polycythemia, for a cut increase in quantity of erythrocytes, leukocytes and thrombocytes, a panmyelosis in marrow, a splenomegaly are characteristic (see the Polycythemia); in hard cases determine the content of erythropoetin, a cut it is increased in most cases at E. and low at a polycythemia.
Pathogenetic reasonable methods of treatment absolute primary E. does not exist. At absolute secondary E. it is necessary to specify the diagnosis of a basic disease in the beginning. Elimination of the reason secondary E. provides its complete elimination.
The symptomatic treatment for the purpose of removal of excess mass of erythrocytes includes bloodlettings 2 — 3 times a week with single-step withdrawal of 400 — 500 ml of blood (all on a course 3 — 4 bloodlettings). At hypoxemic E., caused by inborn heart diseases and hemoglobinopathies, it is necessary to observe extra care with bloodlettings as these E. have compensatory value (the ge-matokritny number is reduced to 52 — 55%, being guided by a condition of the patient; at the same time therapy by oxygen is reasonable). At E., the kidneys caused by defeat or renal transplantation, bloodlettings will see to normalization of an indicator of a hematocrit. It is reasonable to combine bloodletting from a dezagreganta-ma (curantyl, reopo liglyukin), to-rye appoint to the period of therapy bloodlettings and within two weeks after the end of treatment.
Patients with E. need constant dispensary observation:
Bibliography: Demidov A. V. The analysis of the reasons of diagnostic mistakes at a krasnokroviya. Modern approaches to differential diagnosis of an erythremia and secondary hyperglobulias, Rubbed. arkh. * t. 56, No. 6, page 147, 1984; Hereditary anemias and hemoglobinopathies, under the editorship of Yu. N. Tokarev, etc., page 312, M., 1983; The Guide to hematology, under the editorship of,
A. I. Vorobyova and Yu. I. Loriye, page 222, M., 1979; Bert P. La pression barom^t-rique, P., 1878; Donati R. M and. lake, Erythrocythemia and neoplastic tumors, Ann. intern. Med., v. 58, p. 47, 1963;
Hematology, ed. by W. J. Williams a. o., p. 673, N. Y., 1983; Smith J. R. a., Landaws A. Smoker’s polycythemia, New Engl. J. Med., v. 298, p. 6, 1978; Weatherall D. J. Polycythemia resulting from abnormal hemoglobins, ibid., v. 280, p. 604, 1969. BB. H. Tokarev.