Abstract. We report six children, five with acute leukemia and one with aplastic anemia, who during chemotherapy have developed severe aseptic bone necrosis. Although there is no definite proof, our data are highly suggestive of incriminating corticosteroid therapy in large cumulative doses as the main pathogenetic factor.
Haemoglobin fractions and 16 enzymatic activities of red cells of a patient with juvenile chronic myeloid leukaemia are compared to normal, to comparably reticulocyte-rich, non-neonatal and to fetal red cells. The activities of hexoki-nase, triosephosphate isomerase, glyceraldehyde-3-phosphate dehydrogenase, mono-phosphoglyceromutase, enolase and glucose-6-phosphate dehydrogenase are significantly increased in fetal red cells beyond the activities of cell populations with comparable reticulocytosis. The activities of these enzymes are also increased in the patient’s erythrocytes. Together with a haemoglobin F concentration of 54% and a concentration of haemoglobin Bart’s of l% these variations reflect the fetal nature of the red cells. Simultaneously, signs of dyserythropoiesis are found in the red cells of the patient: a very high activity of hexokinase and a low pyruvate kinase activity.
In three young patients with erythroleukemia in whom a partial reversion to the fetal pattern of erythropoiesis occurred there was found additionally an imbalance of globin chain synthesis. The synthesis of beta- plus gamma-chains exceeded that of the alpha-chains. In contrast, physiologic hemoglobin F production occurring in newborn infants and increased hemoglobin F production due to rapidly regenerating erythropoiesis in hereditary spherocytosis and after acute erythroblastopenia are characterized by a well balanced globin chain synthesis. These studies indicate that in distinct cases of juvenile erythroleukemia the genuine reversion to fetal erythropoiesis may be associated not only with a depression of hemoglobin synthesis but also with an imbalanced globin chain synthesis. Unlike adult cases of erythroleukemia without reversion to fetal erythropoiesis the imbalance of globin chain synthesis seems to be a more generalized phenomenon in these cases of juvenile erythroleukemia which is not confined to a particular red cell population.
Twelve children (5 girls and 7 boys, between the ages of 6 and 20 years) in complete remission from previous ALL who had completed their entire anti-leukemic treatment program and who had been off all chemotherapy for at least one year, were included in a study of sleep-related prolactin and gonadotropin rhythms. All the patients had received prophylactic CNS-irradiation. The patients in early puberty showed a sleep-dependent FSH rhythm. Patients in middle-to-late puberty had sleep-related FSH and LH rhythms, and estradiol and testosterone plasma concentrations were normal for their pubertal stage, suggesting recovery of the hypothalamo-pituitary-gonadal feedback system. We conclude that the neuro-endocrine axis is not permanently injured by CNS-irradiation and anti-leukemic therapy.
In 2347 patients fine needle aspiration of the thyroid gland was performed. In 49 cases (2.1%), a final diagnosis of thyroiditis was established. Non-specific granulomatous thyroiditis occurred most frequently (n = 24), followed by lymphocytic hypertrophic thyroiditis Hashimoto (n = 18), focal lymphocytic (n = 5), atrophic lymphocytic (n = 1) and chronic fibrosing thyroiditis (n = 1). Fine needle aspiration biopsy is most suited to diagnose granulomatous thyroiditis. In Hashimoto's thyroiditis cytological investigations are superior to estimation of thyroglobulin antibodies. The cytological method is of little value for the diagnosis of atrophic lymphocytic and chronic fibrosing thyroiditis.
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