The association of HLA-DR5 antigen with longer survival in childhood leukaemia.
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Biomedical subjects
Publications and source records attributed to D Schuler.
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A summary is given of some activities of the Hungarian Study Group for Childhood Leukaemia. Coordinated efforts within the Study Group led to improved therapeutic results. The median survival of patients diagnosed in 1971 was 12 months. For a comparison, out of the 57 patients diagnosed in 1978, 34 are still in their first remission. Experimental work designed to approach the pathomechanism of the disease provided the following main points of interest. 1. Children diagnosed to have L1 type ALL according to the FAB categories fare better than those with L2. 2. ADA activity was essentially normal in patients being in remission and their parents. 3. Steroid receptor determinations can be of value in the planning of therapy. 4. Very low as well as very high initial WBC count indicates a bad prognosis. 5. Children who possess the DR 5 histocompatibility antigen have a better outlook for long complete remissions and cure. 6. Growth hormone secretion and, accordingly, growth rate was normal in the long surviving patients. 7. There was a slight impairment in the performance IQ of children who were under 6 years of age at the time of diagnosis. 8. Emotional disturbances were universal in all patients, but could be much alleviated by regular psychological care and play activity. 9. So far we observed one patient with a second malignancy: his AML manifested 4 years after the successful removal and chemo-radiotherapy of his brain tumour. 10. Children in long, continuous remission-thought to be cured of their disease-possess normal remission lymphocytes with a significantly shorter cell cycle than controls.
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Phytohaemagglutinin-induced leukocyte migration inhibition was studied in 72 children and compared with phytohaemagglutinin skin testing. The methods were used for screening the cell-mediated immune response. The in vitro method, which requires only 10-15% of the number of migrating cells as compared with other migration techniques, proved to be more sensitive, but gave some false-positive results when compared with other immunological parameters. Nevertheless, application of the microdroplet leukocyte migration is advantageous, used either simultaneously with the skin test, or as a single screening method in prematures, mature newborns and young infants, where application and/or evaluation of the skin tests is difficult.
The effect of cranial irradiation with doses of 2,400 and 4,800 rad on the pituitary function of children with acute lymphoblastic leukaemia was studied. The plasma growth hormone level after arginine simulations was normal in 13 out of 15 children. The rise of TSH after TRH stimulation and the metyrapone test were also normal. The growth of 40 children during an observation period of 3 or more years was also normal.
The chromosome mutations and the number of sister chromatid exchanges induced by different kinds of immunosuppressive treatment were investigated in children and adults with certain types of renal diseases. The aim of the study was to find among the treatment schedules those promising good therapeutic results with the least mutagenic effects. A slightly decreased chromosome stability was found in the patients treated by cyclophosphamide therapy.
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391 children received complex chemotherapy according to uniform treatment schedules, proposed by the Hungarian Study Group for Childhood Leukaemia, which was established in 1971. Survival among the patients showed an increasing tendency: more than 50% of patients with ALL are stille alive 3 years after the beginning of treatment. One patient is in complete remission 9 3/4 years after the establishment of the diagnosis. Two types of maintenance therapy were investigated among the patients entered for this study in 1974. "Pulses" with Vincristine-Prednisolone every second month were found to be more optimal than monthly "pulses".
The frequency of acute leukaemia, and the recent results of therapy are described. The requirements to achieve these relatively good results (early and exact diagnosis, intensive therapy, prophylaxis and treatment of side effects, mental and physical rehabilitation) are also discussed.
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A survey is given of the karyotypes observed in 362 children clinically diagnosed as cases of Down's syndrome from whom material was sent to 8 collaborating cytogenic laboratories in Hungary during the period 1965-1974. The sample studied cytogenetically constitutes about 20% of all children born in Hungary in this decade with Down's syndrome. The ways in which patients were selected for cytogenetic examinations could not be specified. In the sample, standard trisomy 21 was found in 91.7%, translocations in 3.9% and mosaicism in 4.4%. The mean age of the mothers of the children investigated was 29.05 years, a relatively low figure which may be explained by the decrease of the mean maternal age over the last decades.
The case of five-year old boy is reported who at the age of 18 months had successfully been operated upon for neuroblastoma and who had subsequently signs of cerebellar encephalopathy. The paraneoplastic conditions of childhood are discussed in connection with the reported case. Opsoclonus was not observed in the patient, and symptoms showed rapid improvement on methotrexate, carmustine and CCNU treatment. Six months later the child was free of neurological disturbances and only displayed a slight mental retardation (IQ: 88). Cytological alterations observed in the CSF during the cerebellar encephalopathy are described in detail. At present, 41 months after the operation the child is well and free of symptoms.
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