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Biomedical subjects

J Lyonnais

Publications and source records attributed to J Lyonnais.

17 recordsLinked to original sources
PubMed1995

Hematopoietic engraftment from a minimal number of apheresis procedures after mobilization of peripheral blood stem cells with chemotherapy and rhG-CSF.

In a cohort of 13 patients, peripheral blood stem cells (PBSC) were harvested by apheresis after mobilization with chemotherapy and rhG-CSF. Nine patients who had excellent mobilization were transplanted with PBSC concentrates from a minimal number of apheresis procedures (mean of 1.5, range = 1-3). During collection, the number of circulating progenitors was on average 50 times higher than those observed at the steady state in the peripheral blood of healthy unstimulated individuals. The mean number of CFU-GM/kg reinfused per patient was 28.1 x 10(4) (range = 18.0-50 x 10(4)). The use of rhG-CSF, at either 1 or 5 micrograms/kg/day, resulted in a significantly greater yield of CFU-GM per mononuclear cells than that observed previously in a comparable group of patients receiving chemotherapy alone. Prompt and durable engraftment occurred after myeloablative chemotherapy. The average duration of absolute neutropenia was 9 days. Transfusion requirements were low with an average of four packed red cell units and two platelet transfusions per patient. The shortest follow-up is 5 months and the longest is 20+ months. The convenience of this new approach to support myeloablative therapy offers new possibilities for the administration of a higher dose-intensity of chemotherapeutic agents. A limited number of apheresis procedures timely harvested will improve the cost effectiveness of transplant programs.

Adult↗
PubMed1992

Hereditary nonspherocytic hemolytic anemia due to pyruvate kinase deficiency: a prevalence study in Quebec (Canada).

Fifty-eight cases of hereditary nonspherocytic hemolytic anemia due to pyruvate kinase deficiency were collected from both laboratories that have performed the assay since 1972 and from attending physicians in the province of Quebec. Using the postal addresses of the probands, a prevalence map was constructed according to the administrative regions of the province of Quebec. The prevalence was found to be higher in eastern Quebec (1/81,838) than in western Quebec (1/139,086). Fifty probands were of French Canadian origin whereas the remaining 6 were recent immigrants.

Anemia, Hemolytic, Congenital Nonspherocytic↗
PubMed1989

[Determination of EGF receptors (epidermal growth factor) in the placenta and breast tumors].

Receptors for Epidermal Growth Factor (EGF) have been identified in different types of tumors and they are often associated with other characteristics which are related to a bad prognosis. A technic has been established to measure membrane receptors in human placenta, which is appropriate for the study of tumors. Preparations of placental membranes were incubated either with increasing amounts (0.1 to 3.0, 10(-9) M) of EGF labeled with 125I (method by saturation) or with 0.2, 10(-9) M labeled EGF and increasing concentrations (0 to 1.6, 10(-9) M) of cold EGF (method by competition). The KD obtained with both technics are not significantly different: 0.85 and 0.77, 10(-9) M respectively, as well as the maximum binding capacity: 5.9 and 4.4 pmol/mg protein respectively. A preliminary study in breast tumors showed that 2 out of 9 contained significant amounts of receptors (58 and 114 fmol/mg protein). One of these two tumors held both estradiol and progesterone receptors. In the other 7 tumors without EGF receptors only one did not contained any estradiol or progesterone receptors. Further studies are needed to evaluate the relationships between EGF/sex hormone receptor status and the degree of breast tumor malignancy.

Breast Neoplasms↗
PubMed1989

Microgranular acute promyelocytic leukemia: a proposed role for a greater deformability of the leukemic cell.

A case of microgranular acute promyelocytic leukemia (APL), M-3 variant, is reported in a boy aged 5 years. The disease, which was rapidly fatal, presented with acute disseminated intravascular coagulation (DIC) and leukocytosis. Different cytomorphologic subtypes of promyelocytes were identified on the basis of cytoplasmic granular patterns: the microgranular type with barely visible cytoplasmic granulations and deeply basophilic cytoplasm and the more characteristic type with large promyelocytes containing azurophil granules. We observed a ratio of large promyelocytes to microgranular promyelocytes of 1:1.2 in the marrow and 1:4 in the peripheral blood. To explain this discrepancy, we hypothesize that the microgranular promyelocytes may be more deformable than the typical promyelocyte and that this intrinsic cellular characteristic may promote marrow egress and increase the likelihood of hyperleukocytosis in the M-3 variant.

Bone Marrow↗
PubMed1988

Thymoma and pancytopenia.

A case of thymoma associated with aplastic anemia is presented. Various therapeutic modalities have been tried and a complete remission finally obtained by antithymocyte globulin.

Adult↗
PubMed1984

Acute Heinz-body anemia due to severe cresol poisoning: successful treatment with erythrocytapheresis.

A patient with massive intravascular Heinz-body hemolytic anemia associated with the presence of bizarre-looking erythrocytes following the oral ingestion of approximately 100 mL of "penetrating oil", a petroleum distillate containing 85% kerosene, 12% cresol and 2% surfactant, is described. He was treated successfully with immediate erythrocytapheresis and forced diuresis.

Anemia, Hemolytic↗
PubMed1979

[Morphology and deformability of erythrocytes in muscular dystrophy].

In recent years, the presence of red cell morphological abnormalities in patients with Muscular Dystrophy has made the object of numerous, often contradictory reports. A possible source of such confusion may lie in the fact that human erythrocytes are extremely sensitive to morphologic transformations resulting from various manipulations or environmental conditions in vitro. We have examined the morphology and deformability of erythrocytes from 7 patients with Duchenne and 9 patients with Steinert (myotonic) Muscular Dystrophy. To avoid preparation artifacts, fresh, unwashed red cells suspended in their own plasma were examined under phase contrast microscopy for the presence of either echinocytes and stomatocytes. Deformability was measured by filtration of dilute cell suspensions at constant flow rate through nucleopore membranes (nominal pore diameter = 3 micrometer). No significant difference was found between the patients' cells and those of 22 healthy volunteer controls. We conclude that previously reported abnormalities may have been the result of preparation artifacts. It appears possible, however, that erythrocytes from Muscular Dystrophy patients may be more sensitive than normal ones to certain stimuli originating from red cell manipulations in vitro.

Erythrocytes↗
PubMed1978

Erythrocyte populations in pyruvate kinase deficiency anaemia following splenectomy. I. Cell morphology.

Light and scanning electron microscopic examination of fresh erythrocytes from the blood of four patients splenectomized for severe pyruvate kinase (PK) deficiency haemolytic anaemia has revealed three morphologically different cell populations. Most striking in these patients was the presence of crenated red cells (30-50% of total) which fit current criteria for definition as echinocytes, but also with unusual surface retraction compatible with the existence of intracellular shrinkage, a phenomenon known to occur after incubation of PK deficient red cells in vitro. Additional cell types include immature reticulocytes (7-8% of total) and thin macrocytic discocytes (45-65% of total) none of which are considered specific for the disease. These morphological findings are in sharp contrast with the paucity of red cell shape abnormalities usually described in PK deficient patients before splenectomy and may provide insight into the mechanisms leading to accelerated erythrocyte destruction in this disease.

Adolescent↗
PubMed1978

Erythrocyte populations in pyruvate kinase deficiency anaemia following splenectomy. II. Cell deformability.

The deformability of three previously described morphological populations of erythrocytes from splenectomized pyruvate kinase (PK) deficient patients was assessed using both Nucleopore filtration and micropipette techniques. Mean deformability was decreased in fresh cells from all patients studied. Selective measurements indicated that immature lobulated reticulocytes in each patient were the least deformable cells, followed by bizarre echinocytes characterized by a hyperviscous behaviour, and by thin macrocytic discocytes whose values were closest to those of control erythrocytes. We conclude that poorly deformable reticulocytes in these patients, after initial trapping in the spleen, are unable to return to the circulation because of their inordinate dependence on mitochondrial oxydative phosphorylation for maintenance of a sufficient intracellular concentration of adenosine triphosphate (ATP); hence their rapid and selective destruction. Splenectomy therefore may be beneficial by allowing such cells to mature more slowly in the circulation of these patients until they develop crenation and hyperviscosity, two manifestations of profound ATP depletion possibly leading to destruction by the liver.

Adolescent↗
PubMed1978

[Morphology and deformability of erythrocytes in muscular dystrophy].

In recent years, the presence of red cell morphological abnormalities in patients with Muscular Dystrophy has made the object of numerous, often contradictory reports. A possible source of such confusion may lie in the fact that human erythrocytes are extremely sensitive to morphologic transformations resulting from various manipulations or environmental conditions in vitro. We have examined the morphology and deformability of erythrocytes from 7 patients with Duchenne and 9 patients with Steinert (myotonic) Muscular Dystrophy. To avoid preparation artifacts, fresh, unwashed red cells suspended in their own plasma were examined under phase contrast microscopy for the presence of either echinocytes and stomatocytes. Deformability was measured by filtration of dilute cell suspensions at constant flow rate through nucleopore membranes (nominal pore diameter = 3 micron). No significant difference was found between the patients' cells and those of 22 healthy volunteer controls. We conclude that previously reported abnormalities may have been the result of preparation artifacts. It appears possible, however, that erythrocytes from Muscular Dystrophy patients may be more sensitive than normal ones to certain stimuli originating from red cell manipulations in vitro.

Adolescent↗
PubMed1976

Hemoglobin Bethesda, beta 145 (HC2) Tyr replaced by His, in a Canadian family.

A hemoglobin mutant with the mobility of hemoglobin (Hb) A on alkaline cellulose acetate or starch gel electrophoresis and mobility between Hb A and Hb S on citrate agar electrophoresis was detected in a patient with erythrocytosis. Structural analysis shows a substitution of histidine by tyrosine at position 145 in the beta-chain.

Adult↗
PubMed1975

Determination of hemoglobin-oxygen affinity on micro samples.

The spectrophotometric determination of hemoglobin-oxygen dissociation on dilute red cell suspensions has been modified by the use of a bicarbonate buffer containing bovine serum albumin. The red cell suspension is equillibrated with known ratios of air and nitrogen and 5 per cent carbon dioxide at atmospheric pressure using two gas pumps. The method enables the hemoglobin-oxygen dissociation curve to be measured on 10 to 20 mul of whole blood, and has given values for the P50 (the oxygen tension at 50 per cent hemoglobin-oxygen saturation) of 25.9 plus or minus 1.5 S. D. which are comparable to the value of 26.0 plus or minus 1.0 S. D. obtained by the mixing technique and by other methods. The addition of bovine albumin (35 mg. per cent or greater) increased the P50 by 3 mm. Hg above that obtained with buffer alone. The P50 value has been compared in bis-Tris and phosphate buffers with and without albumin, and in bicarbonate buffers with the addition of approximately equimolar concentrations of either human or bovine serum albumin or polyvinyl-pyrrolidone (PVP), and buffer alone. The effect of albumin on the P50 value was most marked in bicarbonate buffer, and was statistically significantly greater in the presence of human or bovine albumin than in buffer alone or buffer plus PVP. The addition of albumin could not be explained through an alteration of cell shape or volume, but may be an influence on intracellular pH.

Adenosine Triphosphate↗
PubMedDate not supplied

Anti-PP1Pk and early abortion.

Anti-PP1Pk is found in the serum of individuals of phenotype p. Recurrent first trimester miscarriages have been reported in nearly 50 percent of mothers with the p phenotype. A comparative serologic study of three p phenotype mothers is reported. The proposita had three miscarriages while her two identical twin sisters had normal pregnancies. Following serum fractionation on DEAE-cellulose, serial agglutination with P1 and P2 erythrocytes indicated a possible association between IgG anti-P and the increased risk of abortion.

Abortion, Spontaneous↗