RESUMO
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.
Assuntos
Crise Blástica/sangue , Leucemia Mielogênica Crônica BCR-ABL Positiva/sangue , Leucócitos/metabolismo , Crise Blástica/enzimologia , Glicosaminoglicanos/sangue , Proteínas de Grupo de Alta Mobilidade/sangue , Humanos , Leucemia Mielogênica Crônica BCR-ABL Positiva/enzimologia , Leucócitos/enzimologia , Ornitina Descarboxilase/sangue , Purina-Núcleosídeo Fosforilase/sangueRESUMO
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.
Assuntos
Plaquetas/química , Glicosaminoglicanos/sangue , Hemofilia A/sangue , Leucócitos/química , Doenças de von Willebrand/sangue , Adolescente , Adulto , Separação Celular/métodos , Criança , Eletroforese em Acetato de Celulose , Feminino , Hemofilia A/etiologia , Humanos , Masculino , Doenças de von Willebrand/etiologiaRESUMO
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.
Assuntos
Glicosaminoglicanos/sangue , Leucemia Mielogênica Crônica BCR-ABL Positiva/diagnóstico , Leucócitos/química , Biomarcadores/sangue , Crise Blástica/sangue , Crise Blástica/diagnóstico , Separação Celular , Progressão da Doença , Eletroforese em Acetato de Celulose , Humanos , Leucemia Mielogênica Crônica BCR-ABL Positiva/sangue , Leucemia Mielogênica Crônica BCR-ABL Positiva/patologiaRESUMO
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.
Assuntos
Plaquetas/metabolismo , Glicosaminoglicanos/sangue , Paraproteinemias/sangue , Plaquetas/fisiologia , Eletroforese em Acetato de Celulose , HumanosRESUMO
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.
Assuntos
Eritroblastos/fisiologia , Eritropoese/fisiologia , Glicosaminoglicanos/sangue , Animais , Medula Óssea/efeitos dos fármacos , Células da Medula Óssea , Eritroblastos/efeitos dos fármacos , Transfusão de Eritrócitos , Eritropoese/efeitos dos fármacos , Eritropoetina/farmacologia , Masculino , Policitemia/sangue , Ratos , Proteínas Recombinantes/farmacologia , Estimulação Química , Fatores de TempoRESUMO
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.