Biomedical subjects
E N Thompson
Publications and source records attributed to E N Thompson.
Neonatal colorectal spindle cell sarcoma.
Intestinal spindle cell sarcomas occur very rarely in the neonatal period. There have been 10 previous reported cases. A neonate with colorectal spindle cell sarcoma is reported. The clinical features of the 11 cases and the prognosis of the tumor are discussed.
Serum immunoglobulins to endotoxin core glycolipid: establishment of normal concentrations.
Serum antibody to lipopolysaccharide core glycolipid was measured in normal children and in full term and premature (less than or equal to 1500 g) infants. Antilipopolysaccharide core glycolipid antibody was present in term infants and normal children, and reached adult titres by 15 years of age. The specific anti core glycolipid antibody was predominately of the IgG class. Preterm infants (less than 32 weeks' gestation) had significantly lower titres of antilipopolysaccharide core glycolipid than more mature preterm or term infants. The results suggest that administration of anticore glycolipid immunoglobulin may be beneficial in the treatment or prevention of Gram negative septicaemia in preterm and very young infants.
Serum immunoglobulins to endotoxin core glycolipid: acute leukaemia and other cancers.
Circulating antibody to endotoxin core glycolipid and total serum immunoglobulin concentrations were measured in 86 children with cancer (54 with acute lymphoblastic leukaemia, four with acute myeloid leukaemia, and 28 with various solid tumours). Measurements were made before treatment in the group with acute lymphoblastic leukaemia as well as when patients were both on and off chemotherapy. In the other two groups measurements were made when patients were both on and off treatment. Significant reductions in endotoxin antibody and serum immunoglobulin concentrations were found only in patients with acute lymphoblastic leukaemia. In addition, there was a significant correlation between febrile episodes and the concentration of antibody to core glycolipid in the children with acute lymphoblastic leukaemia. These findings suggest that the use of prophylactic high titre endotoxin antibody may be of benefit to children with life threatening Gram negative infections who are receiving cytotoxic chemotherapy.
Four cases of acute leukemia with trisomy 4.
We report further on four cases of trisomy 4 in acute leukemia. Two cases were FAB type M4, one was type M1, and one was an undifferentiated leukemia. Two of our patients were children, aged 13 months and 11 years, respectively. In one case there was evidence of trisomy 4 occurring as a secondary change.
Parental cancer in an unselected cohort of children with cancer referred to a single centre.
A study of parental cancer in 326 children referred to a single Paediatric Oncology Unit found a significant increase in breast cancer in mothers of children with solid tumours. The 5 tumours found were 8.9 times the expected number. This increase could not be accounted for by any of the known risk factors for breast cancer. The incidence of cancer in mothers of leukaemic children and in all groups of fathers was not significantly raised. Further prospective studies in the mothers of young children with soft tissue tumours are needed to clarify the groups at risk and to determine whether counselling and surveillance of these mothers is appropriate.
Severe combined immunodeficiency syndrome, tissue transplant, leukaemia, and Q fever.
A child born with severe combined immunodeficiency, who was immunoreconstituted by a fetal liver and thymus transplant, developed acute lymphoblastic leukaemia in the donor cell line. During remission she contracted acute Q fever, which gave rise to unexpected complications. Early treatment of the Q fever might have altered the subsequent events and prevented her death.
Oesophageal candidiasis and croup in a child with defective neutrophil motility.
Severe oesophageal candidiasis and croup due to involvement of the larynx developed insidiously in a girl aged 20 months. There had been delayed separation of the umbilical cord and repeated infections associated with a defect of neutrophil motility. The significance of the early clinical features was not fully appreciated and the diagnosis considered only when stricture of the oesophagus became evident. She was treated with oral ketoconazole 100 mg daily. After one month's treatment there was striking radiological improvement apart from the persistence of the oesophageal stricture. The croup resolved completely but there was only partial relief of dysphagia because of the residual stricture. We would emphasis that candidiasis should be anticipated and treated vigorously in children with such a defect of neutrophil motility.
Significance of glucocorticoid receptors and responses in leukaemia.
Glucocorticoid receptor levels and cellular responses to glucocorticoid treatment in vitro were evaluated for the malignant cells from patients with leukaemia. Cells from all cases of lymphoid and myeloid leukaemia contained glucocorticoid receptors; receptor levels and the extent of in vitro responses appeared to depend primarily on cell type and differentiation status. For acute lymphoblastic leukaemia (ALL), high receptor levels were associated with the common ALL phenotype, intermediate levels with null (unclassified) ALL and low levels with T-ALL. Considerable variation in receptor levels and in responses in vitro was observed for acute myeloid leukaemia; receptor levels measured by whole-cell assay did not relate to patient survival.
Effect of leukaemia therapy on neutrophil chemotaxis.
The in vivo effect of various cytotoxic drugs and cranial irradiation on neutrophil chemotaxis was tested in 62 children with acute lymphoblastic leukaemia and in 10 patients with other malignant disease. Cranial radiotherapy had a transient adverse effect on neutrophil chemotaxis after completion of the course which was most marked in children. Methotrexate (MTX) and 6-mercaptopurine (6-MP) alone and in combination had a variable effect of chemotaxis, which was most marked nine days after the end of the course. The effect of 6-MP was clearly dose-related, but continuous therapy (75 mg/m2 day) had the greatest inhibitory effect of all the regimens tested. The in vitro effect was studied in 48 leukaemics and in 85 controls (adults and children); all the patients with leukaemia had been off treatment for at least six months. No difference was found between the effects of drugs tested on control or leukaemic cells. The greatest inhibitory effect was found in vinblastine, adriamycin, 6-MP, and vincristine, all of which were closely dose-dependent, MTX, prednisolone, and asparaginase had no effect on chemotaxis when tested in this way.
Clinical, haematological, and radiological features of children presenting with lymphoblastic mediastinal masses.
During 1968-1978, 68 children presented in six centres in the United Kingdom with lymphoblastic mediastinal masses. The disease was classified as acute lymphoblastic leukaemia (ALL) in 49 children whose bone marrow aspirates contained > 20% lymphoblasts, and as lymphoma (Sternberg lymphosarcoma - LS) in 19 with < 20% marrow infiltration. Male predominated in both groups, and children with ALL had more visceromegaly and lymphadenopathy, lower haemoglobin levels and platelet counts, and higher white cell counts. The most common chest x-ray finding in both groups was a nonspecific anterior mediastinal mass, but the appearances varied considerably and could be classified into three categories, which are illustrated. Pleural effusions were present in 44%. Cell surface-marker studies showed T cell characteristics in 14 of the 17 patients tested. Response to treatment and complications, such as central nervous system (CNS) and testicular relapse, were similar in ALL and LS, and were related to the size of the initial tumour load. Median remission lengths were 37 weeks for ALL and 89 weeks for LS patients. Leukaemic transformation occurred in 47% of LS children. Compared with the outcome in ALL children without mediastinal mass, the results of treatment were poor, regardless of the protocol used, and prophylactic therapy to the CNS reduced the frequency of, but did not eliminate, CNS disease.
Pituitary-gonadal function in children treated for acute lymphoblastic leukaemia.
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Assessment of neutrophil chemotaxis and random migration in childhood. Comparison between leading-front and lower surface count methods.
Neutrophil chemotaxis and random migration were studied in 65 healthy children and 18 normal adults. The method used, the leading-front technique, was more accurate and reproducible than the lower surface count method. Chemotaxis in children under 15 years differed from that in adults. This age effect was most pronounced in those less than 6 years, and particularly in those less than 2 years. When investigating chemotaxis in childhood, comparisons with age-matched controls should be made.
Neutrophil chemotaxis and random migration in acute lymphoblastic leukaemia of childhood. Cellular and humoral aspects.
The cellular and humoral components of the neutrophil chemotactic response were studied in 65 children with acute lymphoblastic leukaemia (ALL). An abnormality in both components was found during relapse. In remission the cellular component only was affected. Although less obvious than during relapse the abnormality persisted while all cytotoxic therapy was given, returning to normal several weeks after treatment had been stopped. The absence of significant infection in relapse in this series could be due to the fact that a remisison was achieved within 3 weeks in all patients. However, a positive correlation between the migration index and incidence of bacterial infection during remission stressed the importance of impaired neutrophil chemotaxis in ALL.
Domperidone or metoclopramide in preventing chemotherapeutically induced nausea and vomiting.
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Disseminated intravascular coagulation in association with congenital neuroblastoma.
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Centres for leukaemia treatment.
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The patterns of fetal haemoglobin production in leukaemia.
Elevated levels of haemoglobin F (Hb F) have been foudn in a wide range of haematological malignancies, but very high levels were found only in juvenile chronic myeloid leukaemia (JCML), and erythroleukaemia occurring in infancy. In both these disorders a reversion to a fetal form of erythropoiesis may occur, as judged by both the structure of the Hb F and by the disappearance of Hb A2 and the carbnoic-anhydrase isozymes during the course of the illness. The clinical picture of JCML is not always associated with a reversion to fetal erythropoiesis; there appears to be a heterogeneity of conditions with this clinical label. Thus the reversion to a completely fetal pattern of erythropoiesis seems to occur in a variety of leukaemias which start in early life. This change is associated with a uniformly bad prognosis. Of a group of 17 patients with acute myeloid leukaemia 15 developed an increase in the level of Hb F about 60 days after the commencement of treatment; significantly greater increases were observed in those achieving a clinical remission. The level of Hb F usually declined during remission but high levels persisted in a few cases. Increased levels of Hb F were found also in patients with other haematological malignancies who had undergone periods of marrow aplasia during treatment. In all cases the Hb F was heterogeneously distributed throughout the red cells. Analysis of gamma15 or gammaCB3 peptides of Hb F from a variety of leukaemias gave glycine compositions ranging from 0.20 to 0.85 residues with many values in the fetal range; all cases with a reversion to fetal erythropoiesis had values in the fetal range. Attempts to confirm the 'fetal' origin of the cells containing Hb F by means of other markers was possible only in the cases of JCML and in one child with erythroleukaemia. These studies indicate that in some forms of leukaemia there may be a genuine reversion to fetal erythropoiesis while in others the emergence of cells containing Hb F appears to be part of a rapid regeneration process occurring after a period of marrow aplasia. The diagnostic and prognostic value of these observations is discussed.