Dermatomyositis and acquired ichthyosis as paraneoplastic manifestations of ovarian tumor.
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Adenocarcinoma↗
Biomedical subjects
L G Tone
Publications and source records attributed to L G Tone.
Translocation (1;5)(q32;q35) in CD30+ anaplastic large cell non-Hodgkin lymphona of childhood. A case report.
The authors studied cytogenetically a case of CD30+ anaplastic large cell non-Hodgkin lymphoma previously diagnosed as malignant histiocytosis and detected a translocation involving chromosomes 1 and 5, t(1;5)(q32:q35). After comparing their findings with those from reports in the literature, they comment about the importance of breakpoint q35 on chromosome 5 and point out the importance of associating morphologic, immunoperoxidase, and cytogenetic findings to confirm the diagnosis of this tumor.
Chromosome Fragility↗
Splenic function in acute leukemia.
Spleen function was evaluated in 36 patients with acute leukemia by enumeration of pitted erythrocytes and analysis of clearance from circulation of heat-damaged 99mTc-labelled erythrocytes (HDE). At diagnosis, the mean percentage of pitted erythrocytes was significantly higher in the patient group (4.04 +/- 7.19%) than in the control group (0.41 +/- 0.35%; p < 0.01), suggesting the occurrence of a splenic hypofunction in a high proportion of the patients. Accordingly, the clearance of HDE showed a pattern of splenic hypofunction in 4 out of 7 patients, based on reduced values for the parameters C20 (overall clearing function of the spleen), K (the rate constant of HDE irreversibly trapped) and K/K1 + K (the fraction of HDE entering the spleen undergoing permanent removal). After successful treatment, the pit counts decreased in all reassessed patients, returning to normal values in 10 out of 12 patients with high pit counts at diagnosis. Additionally, two patients with normal pit counts at diagnosis presented borderline results for the parameters C20 and K, which may suggest a slight splenic hyperactivity in these patients. These results demonstrate, for the first time, the presence of functional spleen abnormalities in patients with acute leukemia.
Acute Disease↗
Growth and sexual maturation of Brazilian patients with sickle cell diseases.
Growth and sexual maturation was studied of 125 Brazilian patients with sickle cell disease whose ages ranged from 7 months to 42 years. Height and weight were significantly lower when compared with the unaffected population. The height and weight deficit increased in the age range of 11-19 years as compared with patients less than 11 years of age. Age of menarche, breast and pubic hair staging in girls, and genitalia staging and testicular volume measurements in boys indicated a delayed sexual maturation for both sexes. The comparison of adult and young patients, however, demonstrated that although puberty is delayed, a normal sexual maturation is attained later in life by most patients.
Adolescent↗
Cytogenetic study of a case of childhood erythroleukemia.
We report a case of childhood erythroleukemia diagnosed by French-American-British Cooperative group (FAB) and by cytogenetic analysis of bone marrow cells. The following major chromosome anomalies were detected: hyperdiploidy with a modal number of 49, three markers consisting of translocations between chromosomes 3, 9, 20, and 15, deletion of the long arm of chromosome 16 (q22----qter), and karyotype instability. These changes were compared with others reported in the literature and discussed in terms of their importance for diagnostic confirmation.
Bone Marrow↗
Liposarcoma of hepatic hilum in childhood: report of a case and review of the literature.
Liposarcoma in childhood has seldom been documented. The rare occurrence of this tumor has resulted in a lack of information about its natural history, prognosis, and management. The differential diagnosis with lipoblastomatosis is obligatory. A 2 year 4 month-old white male child presented at the Hospital das Clinicas of Medical School of Ribeirão Preto, University of São Paulo, with fever, jaundice, and coluria secondary to a tumoral mass of the hepatic hilum causing obstruction of the biliary tree. Histologic and immunohistochemical study at autopsy disclosed a liposarcoma. A literature review found 64 other reported cases. Special attention was given to age, sex, anatomic localization, and histologic aspects of the literature cases. Liposarcoma involving the hepatic hilum and causing obstruction of the biliary tree in childhood was not previously reported in the literature.
Bile Ducts, Intrahepatic↗
Treatment of sickle cell diseases with aspirin.
The effects of long-term aspirin for the treatment of sickle cell disease were compared with placebo in a double-blind trial completed by 29 patients. Each patient was submitted to a 5-month period of treatment with aspirin (median dose 31 mg/kg/day) and an equivalent period with placebo. No clinical or laboratory differences were observed between the two phases, including the frequency of painful crises and infectious episodes, hemoglobin concentration, PCV, reticulocytes, Hb F, bilirubin, irreversibly sickled cells, filterability of red cell, sickling in vitro and hypoxia-induced potassium loss.
Adolescent↗
Hereditary hemoglobin disorders in a Brazilian population.
A survey of hereditary hemoglobin disorders in a mixed Brazilian population of the northeast of the State of S. Paulo revealed a 5.3% incidence of abnormal phenotypes among 400 schoolchildren, 4.5% among 602 mothers and 2.8% among 606 newborns. The most common findings were AS (1.9%), AC (0.8%) and beta-thalassemia (0.8%) heterozygotes, which amount to 3.5% of the sample. In a second selected population of 1,023 patients of the Hematologic Clinic of the University Hospital and their relatives, 471 cases of hemoglobinopathies were detected. The most frequent anomalies were heterozygous beta-thalassemia (35.2%) and Hb S (32.5%), followed by sickle-cell anemia (13.0%), homozygous beta-thalassemia (4.0%) and sickle-cell/beta-thalassemia (4.0%). Other defects detected were delta-beta-thalassemia, Hb C, Hb Hasharon and Hb A2'. One family with alpha-thalassemia has been identified that included a girl with Hb H disease. The significance of these findings is discussed with regard to the racial origin of the population of this region.
Brazil↗
Childhood B lineage acute lymphoblastic leukemia clonality study by the polymerase chain reaction.
PURPOSE: B cell precursors acute lymphoblastic leukemia (ALL) present rearrangements in the heavy chain immunoglobulin and T cell receptor genes, especially in the complementarity determining region 3 (CDR-3) and T cell receptor delta (TCR delta) (V delta 2 D delta 3) regions. These rearrangements may be amplified by the polymerase chain reaction (PCR) and used as clonal markers of B lineage ALL. Our purpose was to study clonality at the DNA level by PCR in B lineage ALL. PATIENTS AND METHODS: Fifty-three pediatric patients (36 with B lineage ALL, 7 with ALL-T, and 10 with nonlymphocytic disease) were investigated using consensus primers for the CDR-3 regions of IgH and TCR delta. RESULTS: Clonality was detected in 86.1% of the patients with B lineage ALL when the primers for the CDR-3 regions were used, in 41.6% when the primers for TCR delta were used, and in 91.6% when the two primers were used together. Biclonality was found in 22.5% and 6.6% of patients that have shown clonality for CDR-3 and TCR delta, respectively. Clonality was not detected in any other samples using these primers. CONCLUSIONS: PCR using CDR-3 and TCR delta primers can be used as an aid for B lineage ALL diagnosis and clonal evolution of theses disease.
Burkitt Lymphoma↗
[Zinc in protein-calorie malnutrition. I. Concentration in serum of children with the clinical types, kwashiorkor and marasmic kwashiorkor].
Zinc concentration was measured in the serum of 10 children with protein-energy malnutrition (eight with clinical signs of kwashiorkor, and two with marasmic-kwashiorkor) on the first, 15th and 30th day after admission. The zinc levels were significantly lower for these patients on the first day than those observed for children with good nutritional status. No significant increase in zinc concentration occurred in the serum of these patients during initial period of recovery of nutritional status. The possibility of zinc supplementation for malnourished children during recovery is discussed.
Child↗