a tumorous systemic disease of hematopoietic tissue in which the impairment of hematopoiesis is manifested by a proliferation of immature pathological cellular elements, both in the hematopoietic organs proper and in other organs (the kidneys, vascular walls, neural pathways, skin, and so forth). The disease is rare (one case per 50,000 population).
Leukemias are grouped into the spontaneous leukemias, the causes of which have not been determined; radiation leukemias, caused by ionizing radiation; and leukemias caused by certain chemical (leukemogenic, or blastomogenic) substances. Leukemogenic viruses have been isolated from a number of animals suffering from leukemia (chickens, mice, rats, dogs, cats, cattle). A viral etiology has not been proved for human leukemia. Leukemias are divided into reticulosis, hemocytoblastosis, myeloid leukemia, erythromyelosis, and megakaryocytic leukemia, depending on cellular morphology (the predominance of particular elements).
Depending on the extent of the increase in the total number of leukocytes and the “flooding” of the blood with immature, pathological cells (normally not found in the blood), leukemias are classified as leukemic, in which the number of pathological leukocytes found in 1 cu mm of blood totals tens of thousands, hundreds of thousands, and even millions, instead of the normal 6,000–10,000; subleukemic, in which the number of pathological leukocytes does not exceed 15,000–25,000 per cu mm; leukopenic, in which the number of leukocytes in the blood decreases but with which pathological, “leukemic” cells may also be present; and aleukemic, in which the number of leukocytes in the blood does not increase and no immature, pathological forms are present. Aleukemic leukemias that progress at a pronouncedly malignant rate are usually called reticuloses.
Leukemias may be acute or chronic. Acute leukemias are marked by a rapid course with a blood picture characterized by an abrupt breakdown of hematopoiesis at a given stage, resulting in the failure of the most immature forms, or blasts, to develop into mature blood cells. The hemogram is marked by some degree of “blastemia,” with an insignificant number of mature leukocytes and an absence of transitional forms. As a rule, acute leukemia occurs with fever, marked anemia, hemorrhagic diathesis, ulcerations, and necrosis in various organs.
Chronic leukemias are classified, according to the aspect of hematopoiesis disturbed, as chronic myelosis (myeloid leukemia), lymphadenosis (lymphocytic leukemia), histiomonocytic leukemia, erythromyelosis, and megakaryocytic leukemia. Chronic myelosis, the most common, is characterized by hyperplasia (proliferation) of the elements of bone-marrow, or myeloid, hematopoiesis, both in the bone marrow proper (the fat marrow of the long bones being replaced by red hematopoietic marrow) and in the spleen (which enlarges significantly), liver, and lymph nodes (where normal lymphoid tissue is replaced by pathological myeloid elements). The blood is flooded with immature, mature, and transitional granular leukocytes.
Chronic lymphadenosis usually follows a protracted but comparatively benign course. The disease develops gradually, chiefly with enlargement of the lymph nodes, although enlargement of the spleen and liver sometimes becomes predominant. Normal myeloid bone marrow is replaced by lymphoid marrow. The blood is flooded with lymphocytes (predominantly mature). Blasts appear during exacerbations. Anemia gradually develops, because lymphoid infiltrates depress the normal hematopoietic function of the bone marrow and because the pathological lymphocytes lose the function of immune competence and manufacture autoaggressive antibodies (in particular, antierythrocytic antibodies, which cause hemolysis). In some cases, the pathological lymphocytes produce antithrombocytic antibodies, which cause thrombocytopenia and hemorrhages. Exacerbations of chronic leukemia are marked by elevated temperature, sweating, wasting, bone pains, increasing general weakness, anemia, and hemorrhagic diathesis.
The pathogenesis (that is, the mechanism of development) of leukemia has not been studied adequately; however, it is believed that the disease begins with cellular mutations that appear in the body after exposure to a variety of exogenous (viral, radiation, chemical) and endogenous leukemogenic agents (in particular, agents that appear with an impairment of tryptophan metabolism). The mutagenic action of these agents disrupts the normal development of the hematopoietic cells, and anaplasia sets in (that is, the loss by the cells of their capacity for normal differentiation and development into mature leukocytes). The accumulation of immature, physiologically deficient cells, manifested in tumorlike growths and leukemic invasion of various organs, results in systemic intoxication and wasting and in dysfunction of the affected organs and systems. Because of the more or less marked decrease in the number of mature, functionally adequate leukocytes and thrombocytes (depending on the form of leukemia and the phase of its development), the course of leukemia is accompanied by a decline in systemic and local immunity. This is manifested in the development of severe infectious, septic, necrotic, ulcerative, and hemorrhagic complications. Leukemic invasion, or metaplasia, of the bone marrow usually gives rise to anemia; this is further aggravated by hemorrhages associated with the thrombocytopenia and intensified hemolysis that result from the autoimmune complications.
Acute leukemia and exacerbations of chronic leukemia are treated in a hospital (preferably one that specializes in blood diseases) where the blood and bone marrow can be monitored. Combinations of cytostatic agents and steroid hormones are used. Sometimes radiotherapy, blood transfusions, supporting and antianemic agents, and multiple vitamins are prescribed. Antibiotics are administered to prevent or control infectious complications. During remissions, acute and chronic leukemia patients receive supporting treatment in specialized hematological departments of polyclinics. In accordance with present regulations in the USSR, all leukemia patients receive all prescribed drugs free of charge.
A. M. LAKTIONOV