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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An unusual presentation of acute megakaryocytic leukemia (AMKL) is reported in two young children. The first child had a 10-day history of ptosis of the right eyelid as the initial manifestation of AMKL, a clinical picture not previously described in this variant of leukemia. Computed tomographic scanning showed multiple intracranial mass lesions, and the diagnosis of AMKL was confirmed by immunophenotyping of bone marrow blasts. The second child had Down syndrome and received alkylating agents and radiation therapy for treatment of metastatic rhabdomyosarcoma of the orbit. She had AMKL as second malignancy. Both patients had acquired chromosome 21 anomalies in their leukemic blasts. The first patient, constitutionally normal, had an i(21q) in his leukemic blasts; the patient with constitutional trisomy 21 had tetrasomy 21 and additional chromosomal changes. The clinical symptoms and the results of morphologic, immunologic, and cytogenetic studies are discussed.
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PMID:Acute megakaryocytic leukemia in children. Clinical, immunologic, and cytogenetic findings in two patients. 183 41

This report describes an exploratory population-based study of maternal and perinatal risk factors for childhood leukemia in Sweden. The Swedish National Cancer Registry ascertained 411 cases in successive birth cohorts from 1973 through 1984 recorded in the Swedish Medical Birth Registry. Using the latter, we matched five controls without cancer to each case by sex and month and year of birth. Mothers of children with leukemia were more likely to have been exposed to nitrous oxide anesthesia during delivery than mothers of controls [odds ratio (OR) = 1.3; 95% confidence interval (CI) = 1.0, 1.6]. Children with leukemia were more likely than controls to have Down's syndrome (OR = 32.5; 95% CI = 7.3, 144.0) or cleft lip or cleft palate (OR = 5.0; 95% CI = 1.0, 24.8); to have had a diagnosis associated with difficult labor but unspecified complications (OR = 4.5; 95% CI = 1.1, 18.2) or with other conditions of the fetus or newborn (OR = 1.5; 95% CI = 1.1, 2.1), specifically, uncomplicated physiological jaundice (OR = 1.9; 95% CI = 1.2, 2.9); or to have received supplemental oxygen (OR = 2.6; 95% CI = 1.3, 1.3, 4.9). Because multiple potential risk factors were analyzed in this study, future studies need to check these findings. We did not confirm the previously reported higher risks for childhood leukemia associated with being male, having a high birth weight, or being born to a woman of advanced maternal age.
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PMID:Maternal and perinatal risk factors for childhood leukemia. 206 25

We have reviewed the records of the 16,564 cases of childhood cancer diagnosed from 1971 to 1983 which were reported to the National Registry of Childhood Tumours in Great Britain for the presence of underlying genetic disease in order to estimate the proportion which results from inherited mutations. A genetic condition was listed for 509 patients, or 3.07% of the total number of tumours. The most frequently recorded diagnoses were: bilateral retinoblastoma (162 cases); Down syndrome (135); neurofibromatosis (90); hereditary Wilms' tumour (71); and tuberous sclerosis (20). The highest hereditary fractions at individual tumour sites were seen for: retinoblastoma (37.2%); kidney (7.2%); leukaemia (2.6%) and brain and spinal cord (2.0%). When information about family history from published reports was incorporated into the figures calculated from Registry data the total genetic fraction was estimated to be 4.2%. We conclude that there is a clear genetic basis for a small minority of the cancers of childhood, but ethnic variation and the lack of known environmental determinants suggest that the total influence of heredity may be higher.
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PMID:An estimate of the heritable fraction of childhood cancer. 206 56

For a better understanding of the karyotype evolution of different marrow cell populations in the course of MDS, 6 patients who eventually developed overt leukemia, belonging to a series of 46 MDS referred to our Institution, were studied ad diagnosis and at leukemic progression. In each case the blast cells were separated from the maturing precursors of the erythroid and granulocytic lineage by centrifugation on a Percoll density gradient. Parallel chromosome investigations were performed in each cell fraction. Cytogenetic analysis performed at presentation did not reveal distinctive karyotype features in metaphases arising in the blast enriched cell fraction, as compared with those obtained from the fraction containing erythroblasts and promyelocytes--myelocytes. These findings suggest that in the initial phase of MDS blast cells may lack distinctive cytogenetic features and may thus represent part of a clonal preleukemic proliferation. At the time of leukemia onset, clonal aberrations [trisomy 21 and del(11)(q23)] showing a restricted pattern of distribution within the blast cell enriched fractions were detected in two patients, whereas one patient showed an increase in size of the abnormal clone carrying monosomy 7, an aberration detected in metaphases obtained from both cell fractions. Thus, some evolutive steps in the natural history of these disorders can be heralded by the acquisition of chromosome aberrations more readily detectable in blast enriched cell fractions. In some cases, partial loss of differentiative capability by the abnormal clone may account for the detection, at leukemia onset, of chromosome aberrations involving both the blast cell fraction and the erythroblast-promyelocyte enriched cell fraction.
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PMID:Chromosome studies of enriched blast cell fractions in myelodysplastic syndromes terminating in acute myeloid leukemia. 209 92

Analysis of the chromosomal changes in various neoplasia is being increasingly carried out not only to evaluate its relationship with the prognosis and biological behaviour of the tumour but also for diagnostic purposes in some cases. Leukaemias are one such group of haematological malignancies which have been most extensively studied in this regard. Karyotypic analysis with Giemsa banding technique was carried out in 35 consecutive cases of Acute Lymphoblastic Leukaemia. Eighteen cases were in children (less than 15 years age) and 17 cases were in adults. M.F. ratio was 1.8:1 FAB classification of these cases showed 31 cases of L1 type and 4 cases of L2 type including one case of T-ALL. Fifteen cases (43%) had no karyotypic abnormality, 6 cases (17%) showed pseudodiploidy, one case each having (-20 + 21), t (9:22), (+ 2-6), (-6 + 8), while two cases had 6 q-.13 cases (37%) showed hyperdiploidy with 6 cases showing trisomy 8 alone, one case (+ 8 + 21), one case trisomy 18, one case + 15(r), one case trisomy 21 plus t (9:22) and one case with trisomy 21 only. Two cases showed more complex abnormalities i.e. + 2 + 8, t (13:22) and -11 + MR + Min + Min. There was one (3%) case of hypodiploidy showing monosomy 6. The above findings are in agreement with studies carried out in other countries except t (13:22) which is rather a scarcely reported abnormality.
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PMID:Chromosomal pattern in acute lymphoblastic leukaemia. 210 33

The outcome in children with acute leukaemia with (n = 90) and without Down's syndrome (n = 4377) was compared. Sixty three (70%) of those with Down's syndrome had acute lymphoblastic leukaemia and in comparison with 3664 (84%) controls had similar prognostic features except for a significant excess of the 'common' immunological subtype of acute lymphoblastic leukaemia. The outcome of the children with Down's syndrome was significantly worse with a five year overall actuarial survival of 28% compared with 59% in the control group. It appeared that both suboptimal chemotherapy and a high rate of infective problems contributed to the poor survival. Twenty six children with Down's syndrome had acute myeloblastic leukaemia and were significantly younger and had a higher percentage of the megakaryocytic and erythroid subtypes of acute myeloblastic leukaemia than the 713 controls. The outcome was similar in the two groups. It is concluded that the patients with Down's syndrome who develop acute leukaemia should receive standard protocols without modification, but aggressive supportive care is necessary to improve outcome.
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PMID:Prognosis of Down's syndrome with acute leukaemia. 213 46

Down syndrome is the most common chromosome abnormality of man. The isolated occurrence of any one of the most of the protean systemic and ocular features of Down syndrome is not specific to the disorder. The associated occurrence of several of these features, however, has distinguished affected individuals as having a distinct entity for nearly 125 years. Recent advances in prenatal diagnosis have allowed the earlier detection, in utero, of chromosomal abnormalities. Although predisposing genetic and environmental influences remain for the most part unknown, advances in molecular biology are leading to a greater understanding of other common disorders that occur with an increased incidence in individuals with Down syndrome; these include Alzheimer's disease, acute childhood leukemia, congenital heart malformations, and immunologic abnormalities. Associated ocular disorders can significantly affect the quality of life of individuals with Down syndrome. As more children with Down syndrome live into adulthood, the ophthalmologist will play an increasing role in allowing them to lead productive and meaningful lives.
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PMID:Down syndrome. 213 46

Two infants with Down syndrome, one 4 weeks old and the other stillborn, at necropsy showed hepatic and pancreatic fibrosis, which was very severe in the liver of the liveborn infant and in the pancreas of the stillbirth. The liveborn infant had typical hematological features of the transient congenital leukemoid reaction of Down syndrome, and the identification of a megakaryoblastic component was consistent with recent opinion that this is a spontaneously-remitting congenital megakaryoblastic leukemia. The hydropic stillborn infant had intense extramedullary megakaryocytosis. The visceral fibrosis may have had a pathogenesis similar to that postulated for the myelofibrosis of megakaryoblastic leukemia in older children.
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PMID:Perinatal visceral fibrosis accompanying the megakaryoblastic leukemoid reaction of Down syndrome. 214 Aug 91

An infant with Down syndrome (DS) and RH isoimmunization developed transient myeloproliferative disorder (TMD) during the neonatal period. At 16 months she presented with acute nonlymphocytic leukemia (ANLL). Cytogenetic studies during TMD showed trisomy 21 only but new abnormalities emerged during ANLL. She is now in complete remission 5 years after diagnosis. Patients with TMD have either trisomy 21 or mosaic 21 in blood and bone marrow but in phenotypically normal children this cell line disappears with resolution of the TMD. A review of the literature indicates that there are no clinical, hematological, or cytogenetic differences between DS children with TMD who subsequently develop acute leukemia and those who do not. However, the leukemia in the former group may differ in presentation, type, and possibly survival time from other DS children who develop leukemia de novo.
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PMID:Acute nonlymphocytic leukemia after transient myeloproliferative disorder in a patient with Down syndrome. 214 49

Transient neonatal myeloproliferative disorders (TMD's) indistinguishable from acute leukaemia by clinical and morphological criteria have been described in neonates with Down's syndrome. To analyse its clinical significance, 10 infants under 1 year of age presenting with Down's syndrome and the morphological picture of acute myelogenous leukaemia were reviewed. 3 of these children had true AML leading to death after 2, 8 and 11 months. In the other 7 children the diagnosis TMD was suggested as spontaneous or in one case interferon-induced remission occurred within 4 to 25 weeks after diagnosis. The interferon-treated patient died of SIDS at the age of 11 months. Another one of the TMD children developed fatal erythroleukaemia at the age of 2 years. Regarding initial clinical and haematological parameters, TMD was indistinguishable from true congenital leukaemie. In all patients classification according to the FAB criteria was difficult, as mainly undifferentiated or poorly differentiated myeloid blasts were seen, sometimes with erythro- or megakaryocytic features. Because of the difficulties in the differential diagnosis of TMD and true AML it is recommended to delay specific cytostatic therapy in neonates with Down's syndrome, until definite progression of the leukaemic process is observed or cytogenetic analyses suggesting true AML are available.
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PMID:[Transient myeloproliferation and acute myeloid leukemia in infants with Down's syndrome]. 214 25


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