[Value of the in vitro culture of granulomonocytic germ cells (CFU-GM) in myelodysplastic syndromes].
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Biomedical subjects
Publications and source records attributed to L Florensa.
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We have studied the amount and intracellular distribution of erythroblastic alkaline and acid phosphatase, nonspecific esterase, and N-acetyl-beta-glucosaminidase in 50 patients with acquired dyserythropoiesis. 19 morphologically normal bone marrow smears served as controls. Alkaline phosphatase was found in all controls (mean 9.4% of erythroblasts). The percentage of positive erythroblasts in pathological conditions varied greatly from absence to over 70%. Acid phosphatase and N-acetyl-beta-glucosaminidase were positive in pernicious anaemia and in acute erythremic myelosis. Nonspecific esterase was only detected in a case of erythremic myelosis. This case also showed a faint metachromasia with the dye azure A.
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A congenital erythrocyte pyruvate kinase (PK) deficiency was found in a 72-year old female patient with chronic myelomonocytic leukemia (CMML). Erythrocyte PK deficiency was associated with an increase in the activity of hexokinase, 6-phosphogluconate dehydrogenase and glutathione peroxidase in erythrocytes as well as a decrease in acetylcholinesterase, glutathione reductase and glucosephosphate isomerase activities. The enzymatic abnormalities were accompanied by alterations in hemoglobin and in i antigen content of erythrocyte membrane. In addition, bone marrow ultrastructural studies showed dyshemopoietic changes in all blood cell lines and especially in erythroblasts. The present findings confirm the close relationship between CMML and acquired dyserythropoietic syndromes and constitute a new observation of the infrequent association of hereditary erythrocyte enzymopathies and leukemia. A survey of the literature is presented.
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