Biomedical subjects
Publications and source records attributed to D Schuler.
Between 1971 and 1981 756 children were diagnosed to have leukaemia in Hungary. Of these, 17 had Down's syndrome. Analyzing the clinical course of their leukaemia revealed a low remission rate and very poor survival in these patients. Down's syndrome seems to present a special high-risk feature, for early death or relapse into leukaemia, which is only partially explained by the accompanying abnormalities.
The late sequelae of leukaemia and tumour therapy are discussed. The most important and/or the most frequent are neuro-psychologic disturbances, deformities of the bones, the decreased reproduction capacity and the second tumours. Because of the rapidly growing number of cured patients a better knowledge of the occurrence, aetiology, prophylaxis and rehabilitation of these changes is needed.
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The treatment of childhood leukemia in Hungary is carried out in the framework of a national multicenter study. During the past ten years 633 newly diagnosed patients were treated in one of the ten centers. Of these, 39 were less than a year old at the time of diagnosis; 24 presented with ALL, 12 with AML, and three were found to have juvenile CML. Initial WBC count, which is the most important prognostic factor in childhood leukemia, was significantly higher in infants than in older children. Accordingly, the treatment results in these patients were inferior to those in children. Therapy of leukemia in infants remains a serious problem and warrants further studies to establish the best approach.
Lymphocyte responsiveness to phytohaemagglutinin and viral antigens was studied in children with Down's syndrome and in controls. Mitogen-responsiveness in the patients was significantly reduced as compared to the control values. Using the lymphocyte transformation test, trisomic patients showed more than a twofold increase in sensitivity to herpes simplex virus as compared to controls. The same test did not show any essential difference between the two groups when adeno- and influenza viruses were used. Immunofluorescence technique, with specifically conjugated antiviral sera, permitted the detection of specific fluorescence in 30% of the patients with Down's syndrome indicating the presence of oncogenic adenovirus type 12 antigen in the circulating lymphocytes. No antibodies--or only very low titres--against adeno- and herpes simplex viruses were demonstrated in the sera of trisomic patients. Mononuclear leukocytes from these patients often showed structural alterations. The incidence of infectious herpes simplex virus and Candida albicans in the saliva of patients was higher than in the control group. It seems that Down's syndrome involves partial disturbance of both the cellular and humoral immune functions--at least with respect to certain viral antigens.
The aim of the study was to establish normal values for TG and TM cells in neonates and small babies who are more susceptible to infections than children and adults. For the same reason, the T- and B-lymphocyte ratios were also determined. The percentage of B- and T-cells in neonates was significantly lower, while their absolute number higher, than the adult value. The percentage of TG cells was significantly higher in neonates than in the other age groups. There was a significant decrease in the ratio of TM cells as compared to adult values. Spontaneous thymidine uptake showed an inverse correlation with age, but the difference was not significant. There was a nearly identical response to PHA in the observed age groups, at least to the single dose of PHA applied.
Children suffering from leukaemia in Hungary are treated according to uniform therapeutic protocols in the framework of a national multi-centre study. Their most important clinical data are stored in the central registry and are analyzed by computerized methods. Since January, 1971, 846 new patients were entered in the registry. Initially treatment results were very poor but showed gradual improvement during the past few years, somewhat parallel to more intensive chemotherapy. The latest treatment protocol includes medium-dose MTX and the combination of ARA-C and VM-26. Preliminary data are encouraging.
Presentation blast cells from 39 children with acute lymphoblastic leukaemia were studied for T- or B-lymphocyte characteristics. Eleven patients had blasts with T-cell phenotype and one child had B-cell leukaemia. The rest belonged to the non-T, non-B group. Clinical features were similar in the T-cell and non-T, non-B cell group, including the initial WBC count and the presence of mediastinal mass. Although patients with T-cell leukaemia fared worse than those in the non-T, non-B group, the difference in survival was not statistically significant. These data are not in accordance with the findings of most other groups and are thought to stem from the chance occurrence of relatively low initial WBC counts in the T-cell group.
The frequency of iron-deficiency anaemia was investigated in 376 children with the aid of serum ferritin determinations. The children, resident in Budapest or Miskolc were 6 months to 3 years old. Marked iron-deficiency (serum ferritin less than 10 micrograms/l) could be demonstrated in 33.8% of the investigated population. The frequency of iron-deficiency was related to the socio-economic and cultural status of the mother, a factor which is thought to influence the feeding habits. In areas where iron-deficiency is frequently detected regular iron-prophylaxis is recommended.
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The psychological and intellectual sequelae of childhood leukemia and its treatment were examined in 48 children with acute leukemia in long remission. Verbal and performance IQ values were determined in addition to full scale IQ. Performance was worse than the verbal IQ in children who were less than six years old at the time of diagnosis. Cranial irradiation even in repeated doses of 2,400 rads had no effect on these children's intellects. Most of the patients, however, showed severe emotional problems when constructing their "world" from given objects. The frequency and severity of these emotional problems could be much alleviated by regular psychological care.
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