bluuebunny/crossref_metadata_2025_split huggingface dataset:
Erythrocyte Pyruvate Kinase Deficiency in Non-spherocytic Hemolytic Anemia: A System of Multiple Genetic Markers?
Authors: ZUELZER WOLF W. , ROBINSON ABNER R. , HSU THERESA H. J. 
Published: October 2019

Abstract
Extreme intrafamilial differences between PK-deficient phenotypes regarding hemolysis, ATP stability, and glucose consumption were observed in two pedigrees in which the index cases had severe nonspherocytic hemolytic anemia. Genetic analysis was consistent with heterozygosity for two distinct interacting mutants in minimally affected relatives of severely anemic homozygotes. Neither the mature erythrocytes of the former nor the reticulocyte-rich cell populations of the latter showed accumulation of glycolytic intermediates, but 2,3-DPG was elevated in both. Despite severe PK deficiency, red cell survival in the minimal type was near normal, glucose consumption was unaffected in three of four subjects tested, and ATP maintenance in vitro was adequate, in contrast to the severe type in which these parameters were grossly depressed.
The genetic and pathophysiologic implications of these findings are discussed. The possibility is considered that defective glycolysis may play a subordinate role in the hemolytic process associated with PK deficiency and that the enzyme defect may be a genetic marker for as yet unknown erythrocytic abnormalities involving an increase of 2,3-DPG and possibly primary membrane lesions creating excessive demands on the energy metabolism of the erythrocytes. Regardless of the mode of gene action, it is concluded that the nonspherocytic hemolytic anemias associated with PK deficiency are genetically and phenotypically heterogeneous, and that the genetic diagnosis cannot rest on PK assay alone.
Studies of Plasma Protein Synthesis by Peripheral Cells from Normal Persons and Patients with Infectious Mononucleosis
Authors: MACKINNEY ARCHIE A. 
Published: October 2019

Abstract
Tissue culture of peripheral blood leukocytes with 14C leucine resulted in radioactive proteins identified as immunoglobulins by immunoelectrophoresis, gel filtration, and immunoprecipitation. Radioactive heterophile antibody was detected by precipitin reaction with heterophile antigen. It was found in significant concentration in infectious mononucleosis cultures but not in normal controls. Apparent synthesis of heterophile antibody and active cell division are two parameters which distinguish infectious mononucleosis cells from normals in vitro.
The Transformed Cell in Thymus-PHA Cultures: Blood or Tissue Origin?
Authors: SCHWARZ M. ROY 
Published: October 2019

Abstract
The present study demonstrated that of the 18 million thymus, lymph node, or spleen cells in cultures containing PHA, on the average only 540, 990, and 6,170 small lymphocytes, respectively, were located in the blood vessels of these organs when the cell suspensions were prepared. Even if all of these cells transformed in response to PHA, it is unlikely that they could account for the transformation observed. Therefore, it was concluded that the lymphocytes of the parenchyma possessed the ability to transform in response to mitogenic agents.
Hemoglobin D Los Angeles in Two Caucasian Families: Hemoglobin SD Disease and Hemoglobin D Thalassemia
Authors: SCHNEIDER ROSE G. , UEDA SATOSHI , ALPERIN JACK B. , LEVIN WILLIAM C. , JONES RICHARD T. , BRIMHALL BERNADINE 
Published: October 2019

Abstract
Data are presented on two Caucasian families with hemoglobin D Los Angeles, (α2β2121gln) In one family, the mother, of Spanish origin, has sickle cell trait and the father, of Mexican origin, has hemoglobin D trait. One child has sickle cell hemoglobin D disease and suffers from a moderately severe hemolytic anemia. In the other family, of English, Scotch, and Irish ancestry, one member has the hemoglobin DAF pattern of hemoglobin D β-thalassemia disease and suffers from moderate hemolytic disease.
Polycythemia and Increased Erythropoietin Production in a Patient with Hypertrophy of the Juxta-Glomerular Appartus
Authors: JEPSON J. , MCGARRY E. E. 
Published: October 2019

Abstract
A patient with proven hypertrophy of the juxtaglomerular apparatus was found to be polycythemic and hypervolemic. Increased levels of erythropoietin were present in the urine and plasma collected from the patient. The associated increase in aldosterone secretion rate, elevated blood renin and angiotensin levels suggest the juxtaglomerular apparatus may directly or indirectly play a role in the production of erythropoietin.
Regulation of Erythropoiesis XXII. Erythropoietin Production in the Newborn Animal
Authors: CARMENA ALBERTO O. , HOWARD DONALD , STOHLMAN FREDERICK 
Published: October 2019

Abstract
Erythropoietin production was examined in the newborn rat. Even though there was significant anemia present during the early neonatal phase of life in the rat, erythropoietin could not be demonstrated in the plasma. Exposure to simulated levels of altitude of 23,000 feet for a period of 18 hr. resulted in the production of demonstrable levels of erythropoietin in the plasma. The amount of erythropoietic activity which could be demonstrated in the plasma of hypoxic animals increased with age from values of 0.76 units/ml. in the 1 day old animal to normal adult values of approximately 8 units/ml. by the thirtieth day of life. Bilateral nephrectomy prior to exposure to hypoxia in the 5 day old and 15 day old rat reduced the amount of erythropoietin to values of 50 per cent and 25 per cent of those observed in the intact hypoxic animal. By the thirtieth day of life, erythropoietin could no longer be demonstrated in anephric animals exposed to hypoxia.
It is concluded that there is a gradual increase in the capacity of the rat to produce erythropoietin in response to an hypoxic stimulus, and that in significant measure during the early neonatal phase, erythropoietin production is accomplished by extrarenal sources.
Satisfactory Red Cell Viability with Slight Excess of Acid Citrate Dextrose
Authors: MAYER KLAUS , D'AMARO JOSEPH 
Published: October 2019

Abstract
Platelet suspensions obtained from blood donors are improved by increased acidification. The simplest way to accomplish this is to collect less blood into the standard quantity of ACD. Since we have previously reported an impairment in red cell viability when blood is collected in a great excess of ACD, it became pertinent to test the survival of stored red cells collected in a "slight" excess of ACD.
The volume of the blood collected was lowered to 375 ml. in 75 ml. ACD (N. I. H. formula A). At this ratio the pH. was 6.5 which is sufficiently low to minimize platelet clumping. The red cells were separated, stored for 21 days at 4 C., and viability was tested by the 51Chromate method. The results showed adequate red cell survival for blood collected and stored in this manner.
Brief Note: Marrow Repopulating Ability of Peripheral Blood Cells Compared to Thoracic Duct Cells
Authors: STORB R. , EPSTEIN R. B. , THOMAS E. D. 
Published: October 2019

Abstract
Ten dogs were exposed to 1200 r. of whole body irradiation at a dose rate of 9.2 r./min. Five of these dogs were then given infusions of 21 to 74 x 109 autologous peripheral blood cells which had been previously stored at -80 C. 4.0 to 19.4 x 109 of these cells were lymphocytes, 0.4 to 4.9 x 109 were monocytes and 16.4 to 50.3 x 109 were granulocytes. All five dogs showed clinical or histologic evidence of bone marrow repopulation. The remaining 5 dogs were given 7 to 22 x 109 autologous thoracic duct lymphocytes. In none of these dogs was marrow repopulation observed.
It was concluded that hemopoietic stem cells are not present in the thoracic duct lymph of the dog in any appreciable number.
Clinical Implications of Cytogenetic Variants in Chronic Myelocytic Leukemia (CML)
Authors: WHANG-PENG JACQUELINE , CANELLOS GEORGE P. , CARBONE PAUL P. , TJIO J. H. 
Published: October 2019

Abstract
Serial cytogenetic studies were performed on 179 chronic myelocytic leukemia patients (CML) at the Clinical Center of the National Institutes of Health. Patients were divided into 6 groups according to cytogenetic findings: Ph1 negative (18 patients), Ph1 negative with other chromosomal abnormalities (3 patients), Ph1 positive (116 patients), Ph1 positive and negative (10 patients), Ph1 positive with other chromosomal abnormalities (24 patients), and Ph1 positive which turned to other chromosomal abnormalities (8 patients). Patients who were Ph1 negative and negative with other chromosomal abnormalities (11.8 per cent of total) had the shortest median survival, 12 to 15 months. In contrast, the median survival of patients in the Ph1 positive groups was 31 to 40 months. The development of other chromosomal abnormalities in Ph1 positive patients presaged the terminal stage of the disease.
The Significance of Bone Marrow Lymphocytosis of Acute Leukemia Patients in Remission
Authors: SKEEL ROLAND T. , HENDERSON EDWARD S. , BENNETT JOHN M. 
Published: October 2019

Abstract
Bone marrow lymphocytosis (BML) 20 per cent or greater occurring during remission of acute leukemia has been looked upon in the past as an unfavorable sign that may presage early subsequent relapse. Seventy-four patients with acute lymphocytic leukemia were studied to evaluate the significance of BML in remission. It was found that 14 patients with less than 20 per cent bone marrow lymphocytes at any time in remission had a median remission duration of 3 months and a median survival of 21 months, while 60 patients with 20 per cent or more bone marrow lymphocytes at any time in remission had a median remission duration of 14 months and a median survival time of 34 months. Patients with AGL and lymphocytosis had remissions and survivals not significantly longer than those without lymphocytosis It is concluded that there is no justification for excluding a patient from complete remission status because of bone marrow lymphocytosis.
Immunochemical Quantitation of Proteins in Single Cells
Authors: GITLIN DAVID , SASAKI TERUO , VUOPIO PEKKA 
Published: October 2019

Abstract
An immunochemical method for the quantitative determination of specific soluble proteins in individual erythrocytes has been described. Application of the method revealed:
1. From 0.5 to 0.9 per cent of the normal adult erythrocytes studied contained small amounts of γ-chains, from 2.5 to 12.5 µµg. as hemoglobin per cell.
2. In cord blood of normal infants of 38 weeks’ gestation, significant numbers of erythrocytes were found which contained either γ-chains or β-chains or both, independently of carbonic anhydrase B.
3. Once initiated within a given erythrocyte, β-chain synthesis in that cell rapidly approached adult rates. Derepression of carbonic anhydrase B synthesis was independent of derepression of hemoglobin synthesis, and in fetal cells which contained carbonic anhydrase B the amount found was well below that of most adult erythrocytes.
4. In 4 patients homozygous for hemoglobin S and in 2 persons heterozygous for thalassemia, an increase in F hemoglobin was associated with an increase in γ-chain content of individual erythrocytes as well as an increase in the number of erythrocytes containing γ-chains; in 2 patients homozygous for thalassemia, an unusual distribution of cells with increased γ-chain content was observed.
Brief Report: Decreased Hemolytic Capacity of Sera From Patients With Paroxysmal Nocturnal Hemoglobinuria
Authors: BLUM STUART F. , GARDNER FRANK H. 
Published: October 2019

Abstract
The sera of patients with Paroxysmal Nocturnal Hemoglobinuria have been shown, in vitro and in vivo, to be less hemolytic for PNH erythrocytes than are normal sera. This defect may result from the liver disfunction encountered in many of the patients although the exact defect has not been defined.
The necessity for using normal sera when performing the acid hemolysis test is emphasized.
Physical Properties of Red Cells as Related to Effects in Vivo. II. Effect of Thermal Treatment on Rigidity of Red Cells, Stroma and the Sickle Cell
Authors: HAM THOMAS HALE , SAYRE RICHARD W. , DUNN REBECCA F. , MURPHY JOHN R. 
Published: October 2019

Abstract
Heating of human or dog red cells at 49 C. for 15 minutes or 60 minutes increased resistance to packing by a centrifugal force, increased viscosity and deformed them. After 15 minutes of heating, osmotic fragility was usually normal but was elevated after 60 minutes with the formation of spheroidal cells.
The increase in viscosity caused by heating red cells at 49 C. for 60 minutes did not appear to result from the effect of heat on the hemoglobin or the hemolysis products of red cells disrupted by sonic treatment. The heating of intact human or dog red cell stroma in serum, or stroma washed multiple times in physiologic saline however, regularly increased their viscosity and produced buds and microstroma. The rigidity of stroma was increased by heating, probably accounting for the increased viscosity of heated intact red cells.
The rigidity of heated stroma of red cells was further demonstrated by showing that the shape changes of the sickling process could be prevented in large part by the heating of sickle cells. Heating did not, however, prevent tactoid formation in stroma-free sickle-cell hemoglobin solutions.
Phagocytosis by Human Eosinophils
Authors: CLINE MARTIN J. , HANIFIN JON , LEHRER ROBERT I. 
Published: October 2019

Abstract
Eosinophils were isolated from the blood of patients with eosinophilia and tested for phagocytic ability. Eosinophils ingested inert polystyrene particles, Escherichia coli, Staphylococcus aureus 502A and Candida albicans, but did so less efficiently than neutrophils. They did not phagocytize antibody-coated red cells. Phagocytosis occurred over a broad pH range and in the presence of inhibitors of oxidative metabolism. Inhibitors of glycolysis, puromycin and colchicine inhibited particle ingestion to a variable extent.
Metabolic concomitants of phagocytosis, including enhanced incorporation of uridine into RNA, stimulation of glucose oxidation and degranulation and loss of lysosomal enzymes were similar in neutrophils and eosinophils. Endotoxin had similar effects on glucose oxidation by eosinophils and neutrophils in vitro.
Brief Report: Ultrastructural Localization of Myeloperoxidase in Human Neutrophil and Rabbit Heterophil and Eosinophil Leukocytes
Authors: DUNN W. B. , HARDIN J. H. , SPICER S. S. 
Published: October 2019

Abstract
Ultrastructural cytochemical observations revealed peroxidase reactivity in primary (azurophil) granules, but not in secondary (specific) granules, of rabbit and human polymorphonuclear leukocytes. Peroxidase reactivity was also observed in the rough endoplasmic reticulum and granules of rabbit eosinophils and in granules of human monocytes.
The May-Hegglin Anomaly: Platelet Function, Ultrastructure and Chromosome Studies
Authors: LUSHER JEANNE M. , SCHNEIDER JOHN , MIZUKAMI I. , EVANS RUTH K. 
Published: October 2019

Abstract
A father and son with the May-Hegglin anomaly were studied. Both were asymptomatic, although the father had a mild thrombocytopenia and a probable platelet thromboplastic function defect. Possible mechanisms for the bleeding tendency observed in approximately one-fourth of the persons with this anomaly are discussed. The autosomal dominant mode of inheritance is again demonstrated, and both father and son were found to have normal chromosomes.
Immunologic Studies of Antihemophilic Factor (AHF, Factor VIII): Cross-Reacting Material in a Genetic Variant of Hemophilia A
Authors: HOYER LEON W. , BRECKENRIDGE ROBERT T. 
Published: October 2019

Abstract
Immunologic studies have identified two genetically distinct types of hemophilia A. While a majority of patients apparently fail to synthesize AHF, a variant has been recognized in which a nonfunctional but antigenically cross-reacting AHF-like protein is present. Plasmas from two of twenty-seven families with hemophilia A have a cross-reacting material which inactivates an anticoagulant to AHF. Nonfunctional cross-reacting material was not present in plasmas from six patients with von Willebrand’s disease.
Deficiency of Vitamin B12-Binding Alpha Globulin in Two Brothers
Authors: CARMEL RALPH , HERBERT VICTOR 
Published: October 2019

Abstract
Persistent deficiency of serum B12-binding alpha-1 globulin was demonstrated in two brothers, manifesting primarily as low serum B12 levels. Despite the inability of one subject to maintain normal levels of B12 in the blood, as shown by persistently low serum values despite monthly injections of B12, no evidence of metabolic B12 deficiency could be found. Tissue B12-storing ability appeared to be intact. His brother exhibited only minimal hypersegmentation of neutrophil nuclei; otherwise, he too presented a completely normal picture.
The normally present alpha globulin B12-binder was virtually absent from saliva and peripheral leukocyte extracts of both subjects.
Current indirect evidence favors neutrophils as at least a partial source of the serum globulin.33 The cause of the possibly hereditary defect in the 2 subjects is unknown. Neither excessive B12-binding protein excretion34 nor a destructive factor in their serum or leukocytes was found.
Brief Report: On the Chemical Abnormality of Hb "Alexandra," a Fetal Hemoglobin Variant
Authors: LOUKOPOULOS DIMITRIS , KALTSOYA ANASTASIA , FESSAS PHAEDON 
Published: October 2019

Abstract
The original fetal hemoglobin variant "Alexandra" has been fingerprinted; the interpretation of the fingerprint is compatible with the substitution of threonine by lysine in the twelfth position of the γ-chain.
The Effect of Bleeding on the Proportion of Red Fluorescing Forms among the Total Normoblasts of Bovine Porphyric Bone Marrow
Authors: RUNGE WALTER , WATSON C. J. 
Published: October 2019

Abstract
The percentage of fluorescing, porphyrin rich normoblasts in the bone marrow of five bovine porphyrics increased after bleeding to a range of 66-91 per cent, as contrasted with 26-50 per cent in the same animals before bleeding. This is believed to be more in accord with a uni- than a bimodal distribution of normoblasts in respect to the genetic abnormality and the resultant overproduction of porphyrin.
Mobility of Human Red Blood Cells of Different Age Groups in an Electric Field
Authors: YAARI A. 
Published: October 2019

Abstract
Using citrated blood of eleven healthy donors the density distribution of red blood cells (D.D.C.) was determined and eight fractions from each sample separated. Separated red cells were resuspended in their own plasma and employed for the electric mobility measurements.
The Ruhenstroth-Bauer Cytopherometer was used at a current of 5 ma. at 24 C. The range of migration of red cells was found to be 0.855 to 1.47 x 10-4cm.2v-1sec.-1. In separated fractions the older the cells the slower they migrated in the electric field.
Brief Report: Hemozygous Erythrocyte Glutathione-Peroxidase Deficiency: Clinical and Biochemical Studies
Authors: NECHELES T. F. , MALDONADO NORMAN , BARQUET-CHEDIAK ANTONIO , ALLEN D. M. 
Published: October 2019

Abstract
An adult Puerto Rican male is described who developed an acute hemolytic episode following the infusion of three units of stored autologous red cells. The acute episode was associated with the presence of numerous red cells containing Heinz bodies. Investigation of his red cells revealed a markedly decreased level of the enzyme glutathione peroxidase. Examination of family members revealed moderately decreased levels of red cell glutathione peroxidase activity in both parents and in a sibling suggesting an autosomal recessive mode of inheritance. Metabolic studies on the erythrocytes from this patient revealed a normal level of hexose monophosphate shunt activity under basal conditions but a lack of normal activation of this pathway in the presence of hydrogen peroxide. The results of these studies support the concept that this enzyme, glutathione peroxidase, plays a major role in mediating the normal red cell response to the presence of peroxides.
The G-Immunoglobulins in Acute Leukemia in Children Hematologic and Immunologic Relationships
Authors: KIRAN OZDEN , GROSS SAMUEL 
Published: October 2019

Abstract
Serial IgG determinations were carried out on 29 children with acute leukemia. With the exception of the pretreatment assays, all determinations were performed during the course of specific antileukemic therapy. Following the initial introduction to cytotoxic agents, a significant, although temporary, reduction in IgG level occurred. The continuous use of antileukemic agents did not alter the IgG levels. Relapses, responsive to changes in therapy, were usually associated with moderate reductions in the IgG levels. Greater reductions in IgG levels occurred following the institution of changes in therapy. In both instances the declines were followed by a return in IgG to the normal range. Declines of significantly greater magnitude and rate occurred in those patients no longer capable of responding to therapy changes. In such patients, levels below 3.8 to 3.5 mg./ml. signified impending death, a lack of response to infection and inability to evoke an IgG response. An example of the interrelationships between clinical and immunologic responsiveness is shown by the patient in complete relapse with a normal IgG assay, who went into remission and had an impressive IgG response following a rubeola infection. Shortly thereafter, her disease process entered an irreversible relapse stage with an IgG level of 2.9 mg./ml., and death occurred within 30 days following a bacterial septicemia.
The relationship between hematologic and immunologic responsiveness raises the provocative question of paradoxically maintaining an immunologically responsive state as a means of prolonging the survival time in the face of persistent immunosuppressive therapy.
Paroxysmal Nocturnal Hemoglobinuria Terminating in Acute Granulocytic Leukemia
Authors: KAUFMANN RETO W. , SCHECHTER GERALDINE P. , MCFARLAND WILLIAM 
Published: October 2019

Abstract
A 58 year old male who developed paroxysmal nocturnal hemoglobinuria and acute granulocytic leukemia is described. This rare occurrence is pertinent in view of recent concepts suggesting common etiologic mechanisms for these diseases.
Brief Report: A Note on Hypertonic Saline Hemolysis of Red Cells
Authors: NESER MADELEINE L. 
Published: October 2019

Abstract
Fine dendritic crystals loaded at their ends with hemoglobin were found to be growing at the evaporating edges of an open hanging-drop preparation of blood diluted with normal saline. In another area dendritic crystals were growing direct from red cells. This phenomenon was found to be reproducible, as also the growth of larger crystals made with stronger NaCl solutions. These structures were identified as atypical NaCl crystals. Large as well as fine dendritic crystals were found still to form after removal of the red cell membranes and stroma and after the removal of plasma together with the remnants of hemolyzed red cells. It was concluded that hemoglobin was the sole agent responsible for modifying the form of the NaCl crystals. It was further apparent that NaCl has a strong affinity even for intracellular hemoglobin, and that hypertonic saline hemolysis of red cells can take place without any shrivelling of the cell membrane, occurring at the site of "membrane defects" located on the cell surface.