[Retrospective analysis of the morphological, cytochemical, cytogenetic, electron microscopic, and biochemical features of blast cells in acute promyelocytic leukemia]. / Retrospektivnyi analiz morfologicheskikh, tsitokhimicheskikh, tsitogeneticheskikh, élektronno-mikroskopicheskikh, biokhimicheskikh osobennostei blastnykh kletok pri ostrom promielotsitarnom leikoze.

The paper presents a retrospective analysis of long-term follow-ups (from 1959 to 2000) of 86 patients with acute pro-myelocytic leukemia, a rare type of hemoblastoses. The specific features of this form of leukemia is that blast cells of the bone marrow and peripheral blood have a specific granularity that plays a decisive role in the development of the severe hemorrhagic syndrome leading to patients' death. The morphological, cytochemical, cytogenetic, electron microscopic, and biochemical features of blast cells in this disease and its pathogenesis, clinical presentation, and treatment are considered. An extract from the records of a female patient who has had a complete clinical and hematological remission for 22 years is given. The follow-up of such a prolonged remission has not been covered in the Russian literature.

[Certain biochemical features of leukocytes in blast crisis of chronic myelogenous leukemia]. / Nekotorye biokhimicheskie osobennosti leikotsitov pri blastnom krize khronicheskogo mieloleikoza.

The content of nuclear high mobility group (HMG) proteins, activities of ornithine decarboxylase (ODC), adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP) and also glycosaminoglycan (GAG) content and composition were studied in leukocytes of patients with chronic myelogenous leukemia in the phase of blast crisis (BC CML). Myeloid and lymphoid cytochemical variants of BC CML differ by biochemical parameters. It is suggested, that the content of HMG-proteins, activities of ODC and PNP, and electrophoretic patterns of GAGs could be used in diagnostics of two main variants of BC CML.

[Leukocyte and thrombocyte glycosaminoglycans in hemophilia A and von Willebrand's disease]. / Glikozaminoglikany leikotsitov i trombotsitov pri gemofilii A i bolezni Villebranda.

AIM: The study of glycosaminoglycanes (GAG) in leukocytes and platelets of patients with hereditary coagulopathy. MATERIALS AND METHODS: GAG concentration, composition and fraction identification were made in 25 patients with hemophilia A and 10 patients with Willebrand disease. RESULTS: In hemophiliacs, leukocytes contained low concentrations of GAG. In those with bleeding and synovitis GAG levels were lower than the average, in those with extensive hematomas in the absence of locomotor disorders the above levels were close to normal. Chondroitinsulphate dominated in GAG composition though it was less polydisperse. Heparin sulphate levels were elevated. Platelet GAG characteristics were close to normal. In Willebrand disease leukocyte GAG content and composition was similar to those in hemophilia A except some differences in electrophoretic properties of small GAG components. CONCLUSION: Metabolism and/or release of GAG from blood cells may be involved in pathogenesis of hemophilia A and Willebrand disease.

[The importance of studies of the glycosaminoglycans in the peripheral blood leukocytes of patients in the diagnosis of the phases of chronic myeloleukemia]. / Znachenie issledovanii glikozaminoglikanov leikotsitov perifericheskoi krovi bol'nykh v diagnostike faz khronicheskogo mieloleikoza.

To elucidate new diagnostic markers of chronic myeloid leukemia (CML) phases, we investigated quantitative and qualitative composition of glycosaminoglycans (GAG) of leukocytes from peripheral blood of 72 patients. Chronic CML phase was characterized by elevated GAG levels (2 times compared to normal values), weakening of anionic properties of chondroitin sulfate (CS) and high amount of heparan sulfate (HS). In CML transformation in the progressive phase overall concentration of GAG grew still higher, GAG fraction composition changed. In the blast crisis there was a sharp fall in the overall GAG, new electrophoretic fractions emerged. In the myeloid variant of the crisis an additional GAG component appeared (GAG-m), whereas in the lymphoid variant another component was found (GAG-1). It is suggested that the number and composition of GAG in peripheral blood leukocytes may serve markers of CML phase.

[The role of glycosaminoglycans and proteoglycans in hemopoiesis and the physiological functions of the blood cells]. / Rol' glikozaminoglikanov i proteoglikanov v gemopoéze i fiziologicheskikh funktsiiakh kletok krovi.

Literature review as well as own data on the role of glycosaminoglycans and proteoglycans of leukocytes, platelets and hemopoietic microenvironment are presented. These compounds are involved in the storage of lysosomal enzymes, cationic antibacterial polypeptides and other granular constituents of blood cells, take part in mediating of cellular interactions, occurring in hematopoiesis, phagocytosis, immunity and other processes. Due to the capacity to react with many substances, including enzymes, cell growth factors, cytokines, receptors, changing their conformation and biological activity glycosaminoglycans and proteoglycans can take part in regulation of cell adhesion, migration, proliferation differentiation and special functions of various types of hematopoietic and lymphoid cells.

[Lysosomal enzyme activity in white blood cells in leukemias]. / Aktivnost' nekotorykh lizosomal'nykh fermentov v belykh kletkakh krovi pri leikozakh.

Total enzyme activity of acidic hydrolases and total neutral proteinase were compared in the post-nuclear fraction of leukocytes from healthy subjects and leukemia patients. The levels of acidic phosphotase and neutral proteinase in lymphoid cells of healthy donors were 11 and 7 times lower than those in myeloid cells, respectively. Patients suffering chronic myeloid leukemia revealed enhanced levels of beta-glucuronidase and neutral proteinases whereas B-chronic lymphoid leukemia involved acidic hydrolase concentrations lower than normal. As chronic myeloid leukemia advanced, neutral proteinase activity dropped dramatically (2.5 times); an aggressive course of B-chronic lymphoid leukemia was accompanied by a 3-fold decrease in acidic hydrolase level. The results may be used as indirect evidence of differences in the role of lysosomal enzymes in the mechanism of protein processing involved in myeloid and lymphoid proliferative pathologies.

[Platelet glycosaminoglycans in paraproteinemic hemoblastoses]. / Glikozaminoglikany trombotsitov pri paraproteinemicheskikh gemoblastozakh.

The content and composition of platelet glycosaminoglycans (GAG) were studied in 24 patients with paraproteinemic hemoblastoses (22 patients with myelomic disease and 2 with Waldenstrom's disease). According to the GAG levels, the patients were divided into 2 groups: 1) 6 patients with its higher levels (181 +/- 8 micrograms of uronic acids per 100 mg of acetone-dried cells) and 2) 18 patients with its close-to-normal levels (129 +/- 4 micrograms per 100 mg of the cells). Marked manifestations of hemorrhagic diathesis were found in 5 patients in Group 1 and in 1 in Group 2. Chondroitin sulfate was demonstrated to prevalent in the platelets of patients with paraproteinemic hemoblastoses, as in those of healthy persons. There was GAG electrophoretic profile depletion due to the reduced number of minor components of non- and low-sulfated GAG in 14 patients. It is suggested that the changes detected in the content and composition of GAG may contribute to platelet dysfunction in these diseases.

[Glycosaminoglycans of erythroblastic islands in the suppression and subsequent stimulation of erythropoiesis]. / Glikozaminoglikany éritroblasticheskikh ostrovkov pri ugnetenii i posleduiushchei stimuliatsii éritropéza.

The GAG types of the rat bone marrow erythroblastic islands were found to be similar to those of human macrophages. Activation of erythropoiesis with the erythropoietin induced an expression of heparin-sulfate.

[The glycosaminoglycans of the bone marrow erythroblast islands in the rat]. / Glikozaminoglikany éritroblasticheskikh ostrovkov kostnogo mozga krysy.

The glycosaminoglycans (GAG) types of normal rat bone marrow's erythroblastic islands were found to be similar to that of human macrophages. Induced anaemia increased the sulphated GAG.

[Interrelation between the ribosome content of lymphocytes and hypogammaglobulinemia in chronic B-cell lymphatic leukemia]. / O vzaimosviazi mezhdu kolichestvom ribosom v limfotsitakh i gipogammaglobulinemiei pri B-kletochnom khronicheskom limfoleikoze.

The serum immunoglobulin levels, ribosome contents and protein synthesis rate in peripheral blood lymphocytes have been studied in 35 patients with chronic lymphatic leukemia. It is shown that in patients whose lymphocyte ribosome content corresponds to that of the donors IgG, IgA, IgM levels were on the average normal whereas in the patients with the decreased ribosome content IgG and IgA were 2-2.7 times lower, while the IgM concentration was normal. Hypogammaglobulinemia of IgG, IgA and IgM types in 47% of patients with the normal ribosome contents was 23%, 29% and 23%, respectively, and in 100% of patients with the low ribosome content it was 72%, 83% and 68%, respectively. There was no distinct correlation between the immunoglobulin levels and the protein synthesis rate. The ribosome pattern in the lymphocyte maturation in chronic lymphatic leukemia is discussed.

[Leukocyte glycosaminoglycans in various forms of myelo- and lymphoproliferative blood diseases]. / Glikozaminoglikany leikotsitov pri raznykh formakh mielo- i limfoproliferativnykh zabolevanii sistemy krovi.

The level and composition of glycosaminoglycans (GAG) were studied in leukocytes of healthy donors and patients with different forms of leukemia. GAG level was found to increase in chronic myeloproliferative disorders. This parameter was far below the norm in most cases of acute leukemia. An evaluation of GAG profile established a relationship between cell GAG level variation, the type of proliferating cells and leukemic transformation. Peculiarities of GAG profile in myeloid and lymphoid cells were identified. It was shown that a study of GAG in leukocytes of leukemic patients may contribute to identification of blast elements and differential diagnosis between leukemia types.

[Glycosaminoglycans in leukocytes in myeloproliferative disorders]. / Glikozaminoglikany leikotsitov pri mieloproliferativnykh zabolevaniiakh.

Content and composition of glycosaminoglycans (GAG) was studied in leukocytes of 30 healthy persons and of patients with different types of myeloproliferative disorders: 32 patients with chronic myeloid leukemia (CML), 10 patients with polycythemia vera (PV), 5 patients with idiopathic myeloid fibrosis (IMF) and 10 patients with acute myelo blast leukemia (AML). The total content of GAG in healthy persons was 97.7 +/- 3.9 micrograms of uronic acid per 100 mg of dry cells, in CML and IMF it was 2-4-fold increased, in PV it was elevated by 20%, in AML it was diminished 3-5-fold. The elevated level of GAG in CML and IMF was due to presence of basophils and considerable quantity of immature cell forms: promyelocytes and myelocytes. In these cells changes in GAGs composition and their properties were shown: an increase of chondroitins deficient in sulfate groups and of heparan sulfate content, especially in basophils. The observed changes in GAG content and composition may be important for biological activity of the leukemic cells.

[Effect of lysosomal acid-soluble proteins on the respiratory activity of the granulocytes of healthy subjects and chronic myeloid leukemia patients]. / Vliianie lizosomal'nykh kislotorastvorimykh belkov na dykhatel'nuiu aktivnost' granulotsitov zdorovykh liudei i bol'nykh khronicheskim mieloleikozom.

A rate of oxygen consumption by granulocytes was measured before cell stimulation and following their stimulation by zymosan in healthy persons and in patients with chronic myelocytic leukemia (CML). The rate of oxygen consumption by granulocytes of donors was higher than that of patients with CML. Addition of lysosomal acid soluble proteins, extracted from normal granulocytes, affected the respiratory activity of stimulated and non-stimulated both normal and leukemic cells, depending upon the protein concentration. The alterations were more marked in granulocytes of donors. Lysosomal acid soluble proteins, extracted from granulocytes of patients with CML did not change the cell respiratory activity.