bluuebunny/crossref_metadata_2025_split huggingface dataset:
Corticosteroid Therapy in Felty's Syndrome and its Effect on Hypersplenic Rats
Authors: Robertson J. H. , Crozier E. H. , Hollinger Mary 
Published: March 2008

Summary. A patient with Felty's syndrome was treated with prednisone with resulting improvement in anaemia. This was due to a decrease in an expanded plasma volume; it occurred rapidly and was not associated with any apparent change in the size of the spleen.A contrasting form of hypersplenism was induced in rats, splenic enlargement being due to storage of methylcellulose in the organ. Corticosteroid treatment of these animals produced haematological changes similar to those in the patient although they occurred much more abruptly. The nature of the changes brought about by steroid therapy was investigated by isotope studies.It is concluded that corticosteroid therapy can improve the anaemia of hypersplenism by reducing an expanded plasma volume and that this effect may occur without parallel improvement in the other haematological changes of hypersplenism. The decrease in plasma volume does not appear to result from a steroid‐induced lymphoid involution and a reduction in splenic size. Instead, it seems likely that this treatment affects an intermediary mechanism by which plasma volume is increased in hypersplenic states. The possible nature of this is discussed.
Plasma Fibrinogen and its Fragments during Streptokinase Treatment
Authors: Gaffney P. J. , Chesterman C. N. , Allington M. J. 
Published: March 2008

Summary. Using immunoprecipitation and sodium dodecyl sulphate (SDS) acrylamide gel electrophoresis, plasma samples were studied from three patients during prolonged streptokinase (SK) infusion (600 000 iu initial dose and 100 000 iu/hr for 3–5 days). During infusion, plasma fibrinogen was degraded to a variety of high molecular weight (mol wt) products, the clottable material from which contained β chains, γ chain dimers, and α chain fragments of about 25 000 mol wt (designation for fibrin sub‐units of the nomenclature subcommittee of the International Committee for Thrombosis and Haemostasis, meeting in Washington, D.C., 1967). The α‐chain polymers (αx) which were present in the fibrin clots before and 14–18 hr after SK treatment, were not present in clots prepared from plasma obtained during SK infusion.Clottable fibrinogen and serum fibrinogen/fibrin degradation products (FDP) were measured and semi‐quantitative estimates of the high mol wt fragments were obtained. After infusion with SK there was a rapid drop in clottable fibrinogen and a rise in some large mol wt fragments. The variable levels of serum FDPs observed and the nearly complete absence of the fibrin α chain polymers (αx) are discussed in relation to the presence of the different types of the high mol wt fragments. The inability of these fibrinogen fragments to clot efficiently or to form α‐chain crosslinks catalysed by factor XIII offers a partial explanation of the haemostatic defect associated with SK infusion.
Abnormal Cells in the Peripheral Blood of Patients with Hodgkin's Disease
Authors: Halie M. R. , Huiges W. , Nieweg H. O. 
Published: March 2008

Summary.The characteristics of large, abnormal cells in the peripheral blood of patients with Hodgkin's disease have been investigated with cytochemical stains. It was possible to distinguish clearly cells of the granulocytic series, megakaryocyte parts, cells of the mononuclear phagocytic system and immunoblasts. These cells, however, are not specific for Hodgkin's disease, for they could also be found in normal subjects, in patients with infectious mononucleosis or cytomegalovirus disease and patients with lympho‐ or reticulosarcoma. Cells considered to indicate dissemination of Hodgkin's disease could only be detected in the peripheral blood of patients with this condition, and not in the other diseases mentioned above. These cells do not react with the periodic acid Schiff stain, Sudan black, peroxidase, non‐specific esterase and sometimes faintly with methyl green pyronin. It is therefore improbable that these cells belong to the categories of the granulocytic or monocytic cells, but a relationship to the lymphoid cells on the one hand or to the reticulum cells on the other could not be excluded on the basis of this morphological investigation.
Lymphocyte Membrane Markers in Acute Lymphoblastic Leukaemia
Authors: Aiuti F. , Papa G. , Lacava V. , Ciarla M. V. , D'Amelio R. , Garofalo J. 
Published: March 2008

Summary. T and B lymphocytes can be detected by specific membrane markers. Spontaneous rosette formation with sheep RBC (E rosette) is a T cell marker, whereas receptors for activated complement component (EAC), for the Fc regions of certain immunoglobulin classes (FcR), and for membrane immunoglobulins (mIg) are specific for B cells. Using these lymphocyte membrane markers we evaluated peripheral blood lymphocytes of 18 patients in different stages of acute lymphoblastic leukacmia (ALL).Our results indicate that in acute untreated ALL there is no set immunologic pattern; instead there is a variety of selective deficiencies. Patients in remission have normal populations of T and B cells, whereas those in relapse present a rather obscure picture.The percentage of lymphocytes with membrane markers was compared to the percentage of blast cells and an inverse relation is observed. This indicates that these are abnormal blast cells.The changes from the untreated acute stage through to remission was followed in one subject, and in two others additional study was performed on bone marrow and spinal fluid lymphocytes.
New Kinetic Role for Serum Ferritin in Iron Metabolism
Authors: Siimes Martti A. , Dallman Peter R. 
Published: March 2008

Summary. The role of the iron in serum ferritin was investigated with regard to iron kinetics. Serum (or plasma) was fractionated on a sucrose gradient by ultra‐centrifugation to separate iron in ferritin from other iron‐containing components in serum. After injection of heat‐treated [59Fe]RBC in rats, detectable labelling of the serum ferritin fraction was brief, between 20 and 40 min. The labelling of other iron components in the serum was maximal between 1 and 2 hr. No labelling of the serum ferritin fraction was detected after intravenous administration of [59Fe]‐transferrin in man and in the rat, or after [59Fe]haemoglobin‐haptoglobin or oral [59Fe2+]citrate in the rat. These results indicate that iron in serum ferritin originates in part from the degradation of haemoglobin in senescent red cells and that this labelled ferritin is cleared from the serum rapidly.In normal rats, [59Fe]ferritin, purified from rat liver and injected intravenously, disappeared from the serum at an exponential rate with a half‐disappearance time (HDT) of 4 min. After 1 hr 97% of the label had accumulated in the liver. After a delay of 24 hr, increasing amounts of label appeared in the RBC to an estimated maximum of 70–80% at 5 days. Conditions which increased or decreased rates of synthesis of haemoglobin altered the percentage of [59Fe]ferritin incorporated into haemoglobin, but these conditions did not change the rate of removal of [59Fe]ferritin from the serum by the liver. These results indicate that the iron in serum ferritin is cleared from the serum much more rapidly than transferrin‐bound iron. The data are consistent with the passage through serum ferritin of a major portion of the iron that is released from the degradation of red cell haemoglobin in the RE system.
Haemoglobin Content of Individual Erythrocytes in Normal and Abnormal Blood
Authors: James V. , Goldstein D. J. 
Published: March 2008

Summary. Procedures were developed for the measurement of the haemoglobin content of individual unstained human erythrocytes in blood smears, using a Vickers M85 scanning and integrating microdensitometer. Possible errors due to instrumental factors were investigated. Spatial distribution error was found to be unimportant when using a flying spot 1.8 μm diameter in the specimen plane. Measurements of an oxyhaemoglobin solution adhered to Bouguer's Law (i.e. absorbance was proportional to pathlength); microdensitometric results obtained at a given wavelength and with a given monochromator bandwidth could be corrected to correspond to λmax and minimal bandwidth by multiplication by an empirical constant.Seventy cells in each of nine control and 10 pathological blood samples were measured. Microdensitometric estimates of the MCH correlated well with those obtained by conventional methods, although the former were slightly higher.The mean coefficient of variation of the haemoglobin contents of the control samples was 14.1%, considerably higher than the instrumental uncertainty (under 4%). Seven of the nine control samples exhibited statistically normal distributions without significant skewness or kurtosis (skewness referring to an asymmetrical distribution around the mean, and kurtosis referring to an excess or deficit of observations in the flanks of the distribution curve); the remaining two samples may not have been completely normal in the biological sense. Seven of the 10 pathological samples had significant skewness and/or kurtosis, and only one pathological sample showed no abnormal parameter (MCH, coefficient of variation, skewness or kurtosis). Bimodal distributions appeared to be present in some cases.Microdensitometry of individual erythrocytes yields information not otherwise obtainable, and may prove valuable in the research investigation of a variety of blood conditions.
An Abnormal Platelet Glycoprotein Pattern in Three Cases of Glanzmann's Thrombasthenia
Authors: Nurden A. T. , Caen J. P. 
Published: March 2008

Summary. A glycoprotein abnormality in membrane fractions isolated from the platelets of patients with Glanzmann's thrombasthenia is described. Glycoproteins solubilized from thrombasthenic and control human platelets have been compared by polyacrylamide gel electrophoresis in the presence of sodium dodecyl sulphate. Whereas in a control human platelet membrane fraction three major glycoproteins of molecular weight 155 000, 135 000 and 103 000 were observed, in a similar fraction from thrombasthenic platelets a glycoprotein of 152 000 molecular weight predominated. No differences were observed in the nature of a high molecular weight acidic glycopeptide released from washed thrombasthenic and control platelet suspensions by trypsin, and the sialie acid content of thrombasthenic platelets was shown to be within the normal range.It is suggested that the glycoprotein abnormality may be due to an altered organization within the thrombasthenic platelet membrane and that further studies are necesasry to investigate the role of membrane glycoproteins in platelet function.
Cation Content and Membrane Deformability of Heterozygous P‐Thalassaemic Red Blood Cells
Authors: Vettore L. , Falezza G. C. , Cetto G. L. , De Matteis M. C. 
Published: March 2008

Summary. The red blood cells of patients heterozygous for 8‐thalassaemia contain less sodium, potassium and water than do normal red cells. Incubation of red cells in plasma at 37°C for 24 hr in the absence of glucose produced the following changes: (1) An increase in the red‐cell sodium content of both normal and thalas‐saemic cells. (2) A decrease in the red cell potassium content which was much more pronounced in thalassaemic than in normal cells. (3) A reduction in the total cation content of the red cells from patients heterozygous for β‐thalassaemia; this reduction seemed to be related to the clinical severity of the disease, as well as the reduction in mean corpuscular volume and osmotic fragility. (4) The trapped plasma values, which reflect the deformability of red cells, were initially greater for thalassaemic red cells than normal cells and in both groups increased after incubation.The addition of glucose to the incubation medium largely prevented the changes produced by incubation. The changes induced by incubation in vitro may be relevant to changes in vivo, where a local lack of glucose might lead to a loss of potassium and water from thalassaemic cells. This could give rise to shrinking of the red cells and a reduction in their deformability which may play a role in the haemolysis of heterozygous β‐thalassaemia.
Quantitation of the ABO System Antigens by Means of Isoprecipitating Antisera
Authors: Pintera J. , Vacl J. , Hrnčířová D. , Kurtinová J. , Zelená E. 
Published: March 2008

Summary. A quantitative method for the determination of blood group specific substances A and B on red blood cells was developed, using them to absorb isoprecipitating antisera anti‐A and anti‐B, followed by estimation of the residual antibody by means of a counterimmunoelectrophoretic technique with standard solutions of A and B substances prepared from saliva. The method was tested on a population of A2B donors.The mean amount of A substance in A2B RBC was 131 × 10−12±217 × 10−12 u/cell in the population of 95 donors. The A substance thus determined agreed significantly with values obtained by classical haemagglutinating titration. The mean amount of B substance in A2B RBC was 222 × 10−12±178 × 10−12 u/cell. The amounts of both the A and B substances were not correlated on the same RBC. Several peaks of the distribution curve of both substances confirmed the nonhomogeneity of the populations, there being a wide standard deviation presumably due to differences in the antigen content of different subgroups.
Investigations on Antithrombin III in Normal Plasma and Serum
Authors: Sas Géza , Pepper Duncan S. , Cash John D. 
Published: March 2008

Summary. Studies on antithrombin III (AT‐III) were made by a modification of the two dimensional crossed immunoelectrophoresis technique and gel filtration. Mixing various quantities of heparin with agarose in the first phase of electro‐phoresis, AT‐III from normal human plasma and serum revealed a heterogeneity which depended on the heparin concentration in the agarose gel. At heparin concentrations higher than 16 u/ml, AT‐III displayed three components with different electrophoretic mobilities. The component with the highest mobility (designated immunoantithrombin III1: IAT‐III1) dominated in plasma. In normal serum, however, the quantity of this component was decreased and the two other peaks with a slower electrophoretic mobility (IAT‐III2 and IAT‐III3) became more evident.Normal human plasma and serum were filtered on Sephadex G‐200 and the AT‐III concentration measured in the fractions by rocket immunoelectrophoresis. The peaks of AT‐III were found in the same fractions for both plasma and serum and were coincident with the albumin peak of the plasma proteins. However, in the case of serum the AT‐III concentration decreased less sharply in those fractions with higher molecular weight than in the corresponding plasma fractions. Analysis of these fractions by crossed immunoelectrophoresis revealed that the two components with slower electrophoretic mobility (IAT‐III2 and IAT‐III3) had higher molecular size than IAT‐III1, that the concentration of IAT‐III2 and IAT‐III3 was significantly higher in serum, and that the high molecular weight components in plasma and serum were qualitatively identical. It is concluded that high molecular weight complexes between AT‐III and activated coagulation factors may be present in normally circulating blood and that their detection and possibly quantitation can be achieved using the heparin/agarose crossed immunoelectrophoresis system.
The Concentration Dependence of the Oxygen Affinity of Haemoglobin S
Authors: May A. , Huehns E. R. 
Published: March 2008

Summary. The effect of the concentration of haemoglobin S (Hb S) on its oxygen‐dissociation properties was studied using either reconstituted Hb‐S cells of different mean corpuscular haemoglobin concentrations (MCHCs) prepared by osmotic lysis, or cells in which Hb S is diluted by the presence of another haemoglobin.Only 4% (phosphate buffer) and 21%(bis Tris) of the low oxygen affinity of fresh Hb‐S cells was found to be due to their slightly elevated intracellular 2, 3‐DPG concentrations since when the cells were depleted of 2, 3‐DPG most of the low affinity remained. The low affinity showed a marked dependence upon haemoglobin concentration which was absent for 2, 3‐DPG‐depleted Hb‐A cells and, by extrapolation, the MCHC at which the oxygen affinities of the Hb‐S cells became identical to that of the Hb‐A cells was 14.5 g/dl in phosphate buffer and 13.1 g/dl in bis Tris.Both fresh and 2, 3‐DPG‐depleted cells containing another haemoglobin as well as Hb S (Hb‐SA, Hb‐SC and Hb‐SF cells) were also found to have low oxygen affinities provided that the intracellular Hb‐S concentration (MC(Hb‐S)C) was above a certain level. These also showed a strong dependence upon the MC(Hb‐S)C. The mean MC(Hb‐S)C at which the low oxygen affinities of the DPG‐depleted cells were abolished were 8.3 g/dl (phosphate) and 11.2 g/dl (bis Tris). Hb F in fresh Hb‐SF cells brought about a much greater increase in oxygen affinity than the same amount of either Hb A or Hb C. In 2, 3‐DPG depleted cells Hb A showed a greater ability to ‘dilute’ the Hb S than did Hb C.The conditions for the low oxygen affinity of Hb S were therefore found to be very similar to those required for the gelling of both pure Hb S, and Hb S in haemoglobin mixtures. It was concluded therefore that the low oxygen affinity of the Hb S was caused by the polymerization and that the difference between the oxygen affinities of Hb‐S and Hb‐A cells may be used as a measure of the polymerization process.
Ethanol Inhibition of Reticulocyte Protein Synthesis: The Role of Haem
Authors: Freedman Michael L. , Cohen Howard S. , Rosman Judith , Forte Frank J. 
Published: March 2008

Summary. Ethanol, in concentrations of 0.05–0.8 M, inhibited intact human and rabbit reticulocyte protein synthesis in the presence of iron‐transferrin for endogenous haem synthesis. Associated with this effect there was a conversion of polyribosomes to monoribosomes and a decreased incorporation of radioactive leucine into nascent globin chains. When physiological levels of ethanol (0.05–0.1 M) were used, these effects were prevented by incubation with 50 μM haemin and reversed by removing the alcohol and reincubating with iron‐transferrin or haemin. The polyribosomal disaggregation was also prevented by stopping ribosomal movement with 5 mM cycloheximide. Neither ATP nor GSH levels were altered in the presence of ethanol. When non‐physiological levels of 0.8 M ethanol were used, haemin did not prevent the inhibition of protein synthesis. Likewise, in the rabbit reticulocyte cell‐free lysate system containing haemin inhibition was noted at concentrations greater than 0.05 M ethanol. The polyribosomal disaggregation in reticulocytes incubated with 0.8 M ethanol was associated with decreased dissociation of monoribosomes into subunits. Similarly, when ribosomes were directly suspended cell‐free in 0.1 or 0.8 M ethanol there was a decreased percentage of subunits.These results indicate that physiological concentrations of ethanol inhibit initiation of reticulocyte protein synthesis secondary to a block in haem synthesis. When intact cells are exposed to high non‐physiological concentrations of ethanol the inhibition is secondary to decreased ribosomal dissociation. The cell‐free lysate inhibition is also through this effect on ribosomal dissociation. This study supports the view that alcohol is a direct toxin to developing red cell precursors via its effect on mitochondrial haem synthesis. The physiological role of the decreased dissociation of monoribosomes into subunits is not yet clear.
Liver Transplantation in Pigs with von Willebrand's Disease
Authors: Bowie E. J. Walter , Woods John E. , Fass David N. , Zollman Paul E. , Owen Charles A. 
Published: March 2008

Summary.Nine pigs with von Willebrand's disease (vWd pigs) received normal auxiliary livers; in the immediate postoperative period, three pigs died, three survived from 3 days to 3 weeks, and three were killed 5–13 weeks postoperatively. There was an increase in factor VIII (up to 90 u/dl) but with a type of factor VIII that was more labile than normal and that decreased after 2 weeks, probably as a result of liver rejection. There was a slight increase in the levels of the ristocetin‐Willebrand factor postoperatively (up to 11%) but this activity was undetectable after 2 weeks. There was no correction of the abnormal bleeding time and platelet retention. When a vWd liver was transplanted orthotopically into a normal pig, the factor‐VIII coagulant activity and the ristocetin‐Willebrand factor remained normal. The abnormality in porcine von Willebrand's disease is only partially corrected by liver transplantation, which suggests that there also is extrahepatic synthesis of the missing or abnormal plasmatic activity or activities.
Changes in Erythroblast Morphology as an Index of Response to Cyanocobalamin in Patients with Megaloblastic Anaemia
Authors: Dawson D. W. , Lewis M. J. , Wadsworth L. D. 
Published: March 2008

Summary.Fifty‐three patients with megaloblastic anaemia treated with cyanocobalamin and folic acid have been studied. Repeat marrow examination was found to be of value in assessing response to treatment. The early improvement in marrow morphology in patients with pernicious anaemia was greater with 1000 μg than with 5 μg doses of cyanocobalamin. The effect of folate deficiency in delaying marrow response to cyanocobalamin in patients with pernicious anaemia is described and combined cyanocobalamin and folic acid treatment was found to be more effective than either alone. The response to large doses of cyanocobalamin in folate deficient patients was unrelated to the initial serum vitamin B12 level.
The Three Transcobalamins in Myeloproliferative Disorders and Acute Leukaemia
Authors: Zittoun J. , Zittoun R. , Marquet J. , Sultan C. 
Published: March 2008

Summary.The unsaturated vitamin B12 binding capacity of whole serum (UBBC) and of the three transcobalamins (TC) has been studied in patients with various haematological diseases including myeloproliferative disorders (MPD) and acute leukaemia. The binding capacity of TC I and TC III was increased in MPD; TC I being particularly high in chronic granulocytic leukaemia (CGL) and TC III especially raised in polycythaemia rubra vera (PRV) and in infectious leucocytosis. The binding capacity of both TC I and TC III correlated with blood neutrophil count and the ratio TC III/TC I was low in CGL and increased in PRV. TC II was increased in acute myelogenous leukaemia, during remission and blast cell crisis of CGL and in refractory anaemia with excess of myeloblasts but not in acute lymphoblastic leukaemia (ALL). TC II correlated inversely with blood neutrophil count. There is an inverse ratio between TC II and TC I at least in myelogenous leukaemia. These abnormalities are discussed in relation to granulocyte kinetics. TC III and TC I reflect probably the total body granulocyte pool and share some biochemical and immunological properties supporting the view that they have a common origin in the more mature stages of the granulocyte cell line while TC II probably originates partly in more primitive granulocytes.
Effects of ADP and ATP on Bovine Fibrinogen‐ and Ristocetin‐Induced Platelet Aggregation in Glanzmann's Thrombasthenia
Authors: Cohen Isaac , Glaser Tova , Seligsohn Uri 
Published: March 2008

Summary.With thrombasthenic platelets, aggregation induced by ristocetin (R) and bovine fibrinogen (BF) is reversible, provided ATP is not added to the system. ADP inhibits BF and R induced aggregation, and when added after the onset of aggregation causes rapid disaggregation. ATP prevents these inhibitory and disaggregation effects of ADP. A mechanism is suggested whereby co‐operative effects occur between receptors in the membranes of thrombasthenic platelets.