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Biomedical subjects

R Berger

Publications and source records attributed to R Berger.

At least 631 records · Page 35Linked to original sources
PubMed1987

Cytogenetic studies on 519 consecutive de novo acute nonlymphocytic leukemias.

Cytogenetic studies were performed in the same laboratory on 519 untreated cases of de novo acute nonlymphocytic leukemia (ANLL) between 1977 and 1985. The overall incidence of clonal chromosome abnormalities was 54.3%; higher in children (67.5%) than in adults (50.4%). The distribution of chromosome abnormalities was uneven, according to the categories of the FAB nomenclature. The highest frequency of chromosome changes was observed in ANLL-M3 and the lowest in M1 and M6. The frequency of specific chromosome abnormalities and of their associated changes were also estimated. Monosomy 7 was detected in three patients with acute megakaryocytic leukemia (M7). Six cases with two abnormal chromosomally unrelated clones were observed and six constitutional chromosome abnormalities were detected. A clearer knowledge of the incidence of various chromosomal changes in ANLL seems necessary for better differentiation between the so-called primary and secondary chromosome abnormalities and for prognostic evaluation.

Acute Disease↗
PubMed1987

Trisomy 4: an entity within acute nonlymphocytic leukemia.

Trisomy 4 is a newly recognized primary chromosome change in leukemia. Five cases of acute nonlymphocytic leukemia (ANLL) are described from the United States and France. As in cases from Belgium, the only chromosome abnormality detected in the leukemic cells was trisomy 4. This was associated preferentially with ANLL of the M4 type (by FAB classification): acute myelomonocytic leukemia.

Acute Disease↗
PubMed1987

Cytogenetic studies on patients with medullary carcinoma of the thyroid.

Cytogenetic studies were carried out on stimulated lymphocyte cultures of patients with medullary carcinoma of the thyroid. Of 32 patients studied, seven had a hereditary carcinoma, and five had multiple endocrine neoplasia type 2A and two had type 2B. Neither a constitutional chromosome abnormality nor an increase in chromosomal breakage were detected in these patients.

Adolescent↗
PubMed1987

Prognostic significance of chromosomal abnormalities in acute nonlymphocytic leukemia: a study of 343 patients.

Clonal chromosome abnormalities of 343 patients with de novo acute nonlymphocytic leukemia (ANLL) have been tentatively correlated with prognosis. All the patients were treated according to therapeutic protocols in the same hospital. The complete remission rate and median survival were generally lower in AA-ANLL (ANLL with only karyotypically abnormal metaphases) when compared with NN- and AN-ANLL. Similarly, AA-ANLL had the poorest prognosis in the majority of the classes of the French-American-British nomenclature. ANLL with inversion and/or deletion of chromosome #16 had the best prognosis, and ANLL with t(8;21) was not particularly favorable, nor was acute promyelocytic leukemia with t(15;17). ANLL with complex chromosomal abnormalities had the poorest prognosis. The conclusion is that chromosomal aberrations do have a prognostic significance in ANLL, but that this significance is dependent on the types of chromosomal aberrations.

Acute Disease↗
PubMed1987

Cytogenetic studies of Sézary cells.

Cytogenetic studies of two patients with Sézary's syndrome and of one patient with mycosis fungoides are reported. One chromosome #14 at band 14q11 was involved in chromosomal rearrangements in one patient. A review of previously reported cases shows that some chromosome abnormalities appeared nonrandomly, involving chromosomes #1, #4, #6, #7, #9, #13, #14, and #17. Abnormalities on 2p and monosomy 10 were emphasized.

Adult↗
PubMed1987

Cytogenetics of Abelson virus-induced malignant lymphoma cell lines in the mouse.

Cytogenetic studies were performed on 11 established murine thymoma or peripheral lymphoma cell lines induced by a thymotropic Abelson virus. In eight cell lines a trisomy 5 was present, whereas, two cell lines had a normal karyotype like fresh tumor cells. In the last cell line a trisomy 11 resulting from a t(5;11) was observed. The origin of this nonrandom chromosome abnormality is briefly discussed.

Abelson murine leukemia virus↗
PubMed1987

Cytogenetic study of myeloid and erythroid colonies in chronic myeloid leukaemia with stable Ph1 mosaicism.

We have investigated two chronic myeloid leukaemic (CML) patients with a stable Ph1 mosaicism to see whether the Ph1 positive cells belonged to more than one lineage. Individual erythroid and granulocytic colonies were cytogenetically examined. Ph1-positive and Ph1-negative colonies were found both in the erythroid and granulocytic lineage supporting the contention that CML with a stable Ph1 mosaicism as well as classical CML involves a pluripotent stem cell.

Bone Marrow↗
PubMed1987

[Chromosome translocations and Epstein-Barr virus in Burkitt's lymphoma].

Burkitt's lymphoma is characterized by particular epidemiological features. It is a frequent childhood tumor in children in tropical Africa and occurs at a much lesser frequency all over the world. Chromosomal translocation affecting the long arm of chromosome 8 (band 8q24) and one of the chromosomes carrying the immunoglobulin loci (chromosomes 2, 14 or 22) are regularly observed in Burkitt's lymphoma, regardless of whether the tumor occurred in high or low incidence areas. The prevalence of Burkitt's lymphoma in Africa appears to be related to two factors: holo- or hyperendemic malaria and presence of Epstein-Barr virus genomes in the tumor cells. We present a model of pathogenesis, in which stimulation of B cells by malaria is the primary event in the development of the disease. The risk of the chromosomal translocation should be increased by increasing the number of new B cells generated per time. According to our model, the translocation leads to constitutive c-myc activation and makes the cells responsive to growth factors without inducing proliferation on its own. Infection of a translocation-carrying cell with EBV may provide an additional growth advantage and drive the cell further towards a fully malignant state.

Burkitt Lymphoma↗
PubMed1987

Urinary excretion of lactate, 2-oxoglutarate, citrate, and glycerol in patients with glycogenosis type I.

Growth retardation and lactic aciduria are well-known abnormalities in patients with a deficiency of either glucose-6-phosphatase or glucose-6-phosphate translocase. In 19 patients with glucose-6-phosphatase and two patients with glucose-6-phosphate translocase, growth retardation was quantified by calculating the height standard deviation score. The urinary excretion of lactate and some other metabolites was quantified by calculating the lactate/creatinine, 2-oxoglutarate/creatinine, citrate/creatinine, and glycerol/creatinine ratios in urine. Significant correlations were found between the lactate/creatinine ratio, the 2-oxoglutarate/creatinine ratio, and height SD score. Urinary lactate appeared to respond promptly to changes of the diet, while urinary 2-oxoglutarate responded only slowly, as did growth itself. The citrate/creatinine ratio and the glycerol/creatinine ratio were within the normal range and varied little. It was concluded that the urinary 2-oxoglutarate excretion primarily reflects the severity of the disease as expressed in stunted growth. Thus, while urinary lactate levels are more suitable for monitoring the diet, urinary 2-oxoglutarate levels can be used as an indication for intensive treatment with hyperalimentation.

Adolescent↗
PubMed1987

Type I tyrosinemia: lack of immunologically detectable fumarylacetoacetase enzyme protein in tissues and cell extracts.

Type I hereditary tyrosinemia is characterized by the almost complete absence of fumarylacetoacetase in tissues and cells from patients. To investigate the nature of the enzyme deficiency, extracts of tissues (liver and kidney) and cells (lymphocytes and fibroblasts) were immunochemically screened for the presence of fumarylacetoacetase enzyme protein. The antibodies used were raised in rabbits against fumarylacetoacetase purified from beef liver. These antibodies cross-reacted strongly with the human enzyme. No cross-reacting material was found in extracts from liver (n = 4) and kidney (n = 1) from patients. Extracts from lymphocytes and cultured skin fibroblasts from patients were investigated as well. However, no cross-reacting material was found in extracts of these cells.

Amino Acid Metabolism, Inborn Errors↗
PubMed1987

T cell-derived blast crisis in chronic myelocytic leukemia.

We describe herein the occurrence of a T cell-derived blast crisis of chronic myelocytic leukemia which presented as a typical T cell leukemia on clinical (thymic mass and high white blood cells) and immunological grounds (T6+ T4+ T8+), with rearranged T cell receptor genes and germ line immunoglobulin genes. A Philadelphia chromosome was detected in all blast cell mitoses. The significance of T cell involvement during the chronic and acute phases of chronic myelocytic leukemia is discussed.

Adult↗
PubMed1987

Reactogenicity and immunogenicity of a recombinant hepatitis B vaccine compared with a plasma-derived vaccine in young adults.

A study of the immunogenicity and reactogenicity of three doses of lot L (10, 20, 40 micrograms), two doses of lot N (10, 20 micrograms) of the SmithKline Biologicals recombinant DNA yeast-derived hepatitis B vaccine and a 20 micrograms dose of the Merck, Sharp & Dohme plasma-derived hepatitis B vaccine was conducted in young adults under randomized, double-blind conditions. Immunization was carried out according to a 0, 1, and 6 month vaccination schedule. Results indicated that the yeast-derived hepatitis B vaccine was well tolerated and highly immunogenic. Reactogenicity to both yeast- and plasma-derived vaccines was mild in severity and low in incidence with no significant differences appearing between the study groups. One month after the third dose, the yeast-derived vaccines induced a high degree of seroconversion ranging between 97.8% and 100%. The response was not lot- or dose-dependent. The administration of the plasma-derived vaccine resulted in an anti-HBs geometric mean titre approximately twice as high as those elicited by the different yeast-derived hepatitis B vaccine doses one month after the third inoculation. However, 11 months following the third dose of vaccine, the anti-HBs titres were similar in all groups. Revaccination of subjects who no longer had detectable anti-HBs one year after the last vaccine dose resulted in an anamnestic response.

Antigens↗
PubMed1987

Transcription of c-myc in human mononuclear cells is regulated by an elongation block.

Human mononuclear cells (MNC) isolated from tonsils express very low amounts of c-myc RNA. High expression of the c-myc gene is achieved by stimulation of MNC with pokeweed mitogen (PWM). Nuclear run-on transcription experiments revealed activation of the c-myc gene on the transcriptional level. In unstimulated cells a high transcriptional activity is observed on exon 1, but not on exon 2 and 3. After PWM stimulation, however, a high transcription rate is detected on the entire c-myc gene. Therefore, we conclude that c-myc gene expression in MNC is regulated by an RNA elongation block within the first exon. The analysis of the c-myc chromatin structure revealed that DNAaseI hypersensitive site II upstream of the gene is present in unstimulated as well as stimulated MNC, indicating that accessibility of this site is an essential but not sufficient requirement for c-myc gene expression.

Cell Nucleus↗
PubMed1987

[Acute myelomonocytic leukemia with abnormal eosinophils].

A total of 32 patients with acute myelomonocytic leukemia and abnormal bone marrow eosinophils (LAM4 Eo) have been treated at this Institute since 1977. Abnormalities of chromosome 16 have been noted in 14 of 22 evaluable cases. After a median follow-up of 4 years, median duration of complete remission was 46 months and median duration of survival had not been reached; 8 patients have been receiving no therapy for between 1 and 6 years. These results compare favorably with LAM4 without abnormal bone marrow eosinophils (median duration of complete remission: 12 months; median duration of survival: 14 months). LAM4 Eo constitute a particular entity, with a special hematological presentation, the incidence of abnormalities of chromosome 16, and a particularly good prognosis.

Adolescent↗
PubMed1987

Effects of granulocyte-macrophage colony-stimulating factor and erythropoietin on leukemic erythroid colony formation in human early erythroblastic leukemias.

Erythroid colonies from five patients with an early erythroblastic leukemia were obtained in "serum-free" cultures in the presence or absence of recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF) and homogeneous native erythropoietin (Epo). Erythroid colonies with abnormal morphology and karyotype could be grown in different culture conditions. Their erythroid nature was ascertained by the presence of carbonic anhydrase I and glycophorin A. Leukemic erythroid progenitors strongly differed from normal progenitors in that spontaneous colonies were always obtained, sometimes with an extremely high plating efficiency (up to 5.7%). Colonies were found to be autonomous from exogenous hematopoietic growth factors because they were still obtained with a high plating efficiency at an average of one cell per culture in the absence of any added growth factor. No evidence for an autocrine secretion of Epo or GM-CSF emerged because Epo or GM-CSF could not be detected by biologic or radioimmunologic assays from the culture supernatant or cellular extracts of the leukemic cells and that Epo or GM-CSF antibodies did not block autonomous growth. In all cases, however, hematopoietic growth factors increased the plating efficiency of the abnormal erythroid progenitors. In the two "de novo" leukemias, leukemic erythroid progenitors responded primarily to Epo, whereas in the three other patients' (chronic myeloid leukemia) blast crisis they responded maximally to GM-CSF plus Epo. Recombinant erythroid-potentiating activity had no effect in any of these cases. These results suggest that the leukemic erythroid clonogenic cells arise from expansion of erythroid progenitors at different levels of differentiation (ie, CFU-E or BFU-E, depending upon the disease) and that autonomous growth is not related to a secretion of Epo or GM-CSF.

Cell Cycle↗
PubMed1986

A t(8;14)(q24;q11) translocation in a T-cell leukemia (L1-ALL) with c-myc and TcR-alpha chain locus rearrangements.

Cell lines established from T-cell leukemias have recently been reported to exhibit a chromosome translocation t(8;14) involving proto-oncogene c-myc and the gene of the T-cell receptor alpha-chain(TcR-alpha). In this work, we have studied a case of T-cell leukemia presenting a t(8;14)(q24;q11) translocation that was found in fresh leukemic cells taken during relapse, but was absent in cells collected at diagnosis. Hybridization analysis showed that the breakpoint on chromosome 8 was located 3' to the c-myc exon 3. A TcR-alpha-specific original probe (D14S7, Mathieu-Mahul et al., 1985) revealed two differently rearranged patterns in DNA from leukemic cells obtained at diagnosis and during relapse. In contrast, the rearranged TcR-beta-gene DNA pattern did not change during the course of the disease, indicating that leukemic cells were clonally related. These data indicate that the chromosome breakpoint in 14q11 is situated in the TcR-alpha locus. These results suggest that translocations t(8;14) involving TcR-alpha and c-myc genes in T-cell malignancies are analogous to variant t(2;8) and t(8;22) translocations observed in Burkitt lymphoma. They also establish that the same types of molecular rearrangements due to a t(8;14)(q24;q11) translocation, at first described in T-cell lines established in culture, also exist in vivo and may play a role in the evolution of the leukemic process.

Child↗