bluuebunny/crossref_metadata_2025_split huggingface dataset:
Expression of a chronic granulomatous disease-like defect by fluoride- exhausted neutrophils
Authors: Matzner Y , Brass LM , McMurrich BJ , Peters WA , Andre-Schwartz J , Babior BM 
Published: October 2019

Abstract
Neutrophils incubated with 20 mM F- express a respiratory burst without degranulating or performing phagocytosis. After 60 min of F- treatment, the burst is exhausted and cannot be restarted. Neutrophils so treated have a microbicidal defect similar to that of chronic granulomatous disease (CGD): they kill Str. mitis at a nearly normal rate, but show a marked impairment in the destruction of S. aureus. They differ from CGD neutrophils in that they also display a defect in motility. This defect, however, is not so severe as to seriously impair their ability to kill bacteria by mechanisms that are independent of endogenously generated microbicidal oxidants.
The clearance of 131I-human plasma ferritin in man
Authors: Worwood M , Cragg SJ , Williams AM , Wagstaff M , Jacobs A 
Published: October 2019

Abstract
Ferritin was purified 33,000-fold from the plasma of patients with idiopathic hemochromatosis. The plasma ferritin was labeled with 131I and injected into 2 normal men. Clearance was found to be relatively slow, with 50% 131I-ferritin remaining in the plasma at 27–30 hr. The fraction of plasma ferritin that bound to concanavalin-A was found to be cleared more slowly than the nonbinding fraction. These findings confirm our previous suggestion that glycosylation is a major factor prolonging the survival of ferritin in the plasma, but differ from the results of earlier studies in experimental animals and preterm infants, which indicated very rapid plasma ferritin turnover.
Modulation of murine erythropoiesis in vitro by syngeneic thymocytes: interactions of enhancing and suppressing subpopulations with fluorescent anti-theta antibody and polyamino acids
Authors: Sieber F , Sharkis SJ 
Published: October 2019

Abstract
As we have shown previously, cocultures of bone marrow cells with large numbers of syngeneic thymocytes enhance erythroid colony formation in plasma clots, whereas cocultures with low numbers of thymocytes suppress erythroid colony formation. In this article, we present evidence that the enhancing and suppressing functions of thymocytes are most likely mediated by at least two separate subpopulations. When thymocytes were fractionated on the basis of cell surface density of the theta antigen, enhancing cells were limited to the high theta density fraction, whereas suppressing cells were accumulated in the low theta density fraction. When thymocytes were fractionated on the basis of negative surface charge, the enhancing cells were recovered among the more negatively charged cells. A short in vitro incubation with polyamino acids selectively abrogated the suppressor function.
Studies on the effect of deoxyadenosine on deoxycoformycin-treated myeloid and lymphoid stem cells
Authors: Aye MT , Dunne JV , Yang WC 
Published: October 2019

Abstract
Adenosine deaminase (ADA) deficiency has been reported in association with severe combined immunodeficiency disease (SCID). The mechanism by which ADA deficiency causes immune dysfunction has been investigated in model systems to which the ADA inhibitor deoxycoformycin (dCf) had been added. Previously, we demonstrated that dCF did not prevent proliferation and differentiation of myeloid and lymphoid stem cells. We have now shown that addition of deoxyadenosine to dCf-containing cultures inhibited proliferation of hemopoietic stem cells. This inhibition was, however, equally effective for both normal myeloid and lymphoid stem cells. These findings suggest that other differences may exist between SCID myeloid and lymphoid stem cells to account for the relative sparing of myelopoiesis in SCID patients.
An investigation into the role of coagulation factor XIII in ADP- induced aggregation and fibrinogen binding with rabbit platelets
Authors: Harfenist EJ , Raychaudhuri G , Packham MA , Mustard JF 
Published: October 2019

Abstract
Because there was a possibility that activated factor XIII (factor XIIIa) might stabilize a platelet-fibrinogen aggregate through its crosslinking action, we have isolated plasma factor XIII, activated it, and studied the effect of factor XIIIa at a concentration of 3.3 micrograms/ml on aggregation and 125I-fibrinogen binding of rabbit platelets stimulated with 9 microM ADP. Factor XIIIa did not cause aggregation in the absence of ADP, nor did it enhance ADP-induced aggregation or substantially stabilize the platelet aggregate. The presence of factor XIIIa did not affect the amount of fibrinogen bound to platelets immediately after stimulation with ADP, but it appeared to cause a slow specific binding of 125I-fibrinogen to platelets whether or not they were stimulated with ADP. This binding, which was not inhibited by prostaglandin E1, did not lead to aggregation and was accompanied by crosslinking of fibrinogen through its A alpha and gamma chains, either to other fibrinogen molecules or to a platelet protein or proteins.
Effect of heparin on the inactivation rate of human factor XIa by antithrombin-III
Authors: Scott CF , Schapira M , Colman RW 
Published: October 2019

Abstract
Factor XIa catalyzes an important reaction in the early phase of blood coagulation by converting factor IX to an active enzyme (factor IXa). Although antithrombin-III, an inhibitor of factor XIa, normally accounts for only one-sixth of the plasma inhibitory activity against factor XIa, its effectiveness has been reported to be enhanced by heparin. We have reinvestigated the ability of heparin to potentiate factor XIa inhibition by both purified antithrombin-III and plasma using synthetic tripeptide amide substrates as well as a coagulant assay. No increase in the inactivation rate of factor XIa amidolytic activity by purified antithrombin-III was observed in the presence of therapeutic heparin concentrations (1 U/ml), although inhibition of the amidolytic activity of thrombin by purified antithrombin-III was enhanced at least 20-fold by the same concentration of heparin. Furthermore, despite the ability of heparin (1 U/ml) to increase the inactivation rate of thrombin by plasma, no acceleration of the rate of inhibition of factor XIa by plasma was observed. Similar results were found when the inhibition of factor XIa was monitored with a coagulant assay after first removing the heparin. Only at heparin concentrations of 5 and 10 U/ml, was a 2- and 4-fold increase in the inactivation rate of factor XIa by purified antithrombin III observed. Therefore, in both purified systems as well as plasma, heparin, at concentrations observed in clinical practice, does not accelerate the inactivation rate of human factor XIa by antithrombin-III.
Aneuploidy and percentage of S-phase cells determined by flow cytometry correlate with cell phenotype in childhood acute leukemia
Authors: Look AT , Melvin SL , Williams DL , Brodeur GM , Dahl GV , Kalwinsky DK , Murphy SB , Mauer AM 
Published: October 2019

Abstract
Cellular DNA content distributions of propidium-iodide-stained bone marrow blasts were determined by flow cytometry (FCM) for 225 untreated children with acute leukemia and were correlated with leukemia cell phenotype and karyotype. Aneuploidy of the primary malignant stem line was detected in 54 cases (24%): 51 hyperdiploid and 3 hypodiploid. A second stem line with approximately twice the DNA content of the primary stem line was recognized by FCM in 28 cases (23 ALL, 5 ANLL) and may be an important source of leukemia cell heterogeneity. The degree of DNA content abnormality detected by FCM was highly correlated (r = 0.98) with the number of whole chromosome gains or losses in the leukemia karyotype. Aneuploidy detectable by FCM was more frequent in acute lymphoblastic leukemia (ALL) (52 of 173, 30.1%) than in acute nonlymphoblastic leukemia (2 of 52, 3.8%) (p less than 0.001). In the ALL group, aneuploidy was significantly correlated with the cell surface expression of common ALL antigen: 46 of 127 antigen-positive cases were aneuploid compared to 6 of 46 antigen-negative cases (p less than 0.003). Only 2 of 21 cases of T-cell ALL without common ALL antigen had detectable aneuploidy, which was significantly less than in the common ALL group (p = 0.02). The median percentage of cells in S- phase was significantly greater for B-cell and erythrocyte rosette- positive T-cell ALL, than for the other phenotypic subgroups. We conclude that aneuploidy and S-phase cell percentage are correlated with the state of leukemia cell differentiation. The biologic basis for the correlation is not established, but may be linked to the process of malignant transformation.
Anemia of spaceflight
Authors: Tavassoli M 
Published: October 2019

Abstract
A consistent deficit in the red cell mass has been observed during both the American and Soviet orbital space flights and is sometimes referred to as “astronaut anemia.” This may be associated with a reduction in plasma volume so that the hematocrit and the hemoglobin concentration remain unchanged. During the Gemini program, the hypobaric hyperoxic atmosphere of the spacecraft led to oxidative injury to the red cells, causing hemolysis. Thus, the atmosphere proved to be, in part, responsible for the deficit. However, a similar deficit of a lesser magnitude was again observed in subsequent flights with normal ambient PO2 as well as in the Soviet flights in which an atmosphere essentially of see level air is used. The cause of this deficit seems to be suppression of erythropoiesis, as indicated by reticulocytopenia and erythroid hypoplasia of the marrow. No suppression of erythropoiesis has been observed in ground-based experiments carried out under almost identical conditions. Thus, the suppression of erythropoiesis is thought to be related to weightlessness. The reason for the suppression is not known but may be related to total inhibition of bone formation.
The clinical significance of the morphological subdivision of diffuse "histiocytic" lymphoma: a study of 162 patients treated by the Southwest Oncology Group
Authors: Nathwani BN , Dixon DO , Jones SE , Hartsock RJ , Rebuck JW , Byrne GE Jr , Sheehan WW , Kim H , Coltman CA Jr , Rappaport H 
Published: October 2019

Abstract
We grouped 162 patients wtih advanced, diffuse histiocytic lymphoma (DHL) into various morphological subtypes to ascertain whether there were any significant differences in survival among them. These patients were staged and treated from 1972 to 1977 according to the protocols of the Southwest Oncology Group. Of the 159 patients on whom a consensus on the diagnosis was reached, 115 were classified morphologically as having large non-cleaved, 26 as B-immunoblastic, 9 as large cleaved, and 6 as T-immunoblastic. The 3 remaining patients did not fit any of these subtypes, but each had a single prominent nucleolus in most tumor cells (“prominent nucleolus” type). Morphological subdivision of DHL did not identify any subgroup of patients with a significantly longer survival, but clinical parameters such as stage, symptoms, and type of treatment significantly influenced survival times.
Characterization of the catalytic subunit of factor XIII by radioimmunoassay
Authors: Skrzynia C , Reisner HM , McDonagh J 
Published: October 2019

Abstract
Plasma factor XIII is composed of two subunits, a and b, whereas platelet and other intracellular zymogens have only a-subunits. The catalytic subunit, a, is the same in all forms. In order to characterize the interactions of 1- with b-chains in the plasma zymogen and a-chains with other molecules and to correlate factor XIII activity with a-protein, a specific, sensitive radioimmunoassay was developed. With the polyclonal antisera used, the assay recognizes all molecular forms of a (zymogens, activation intermediates, enzyme) equally well. The assay can be used to determine a-chain concentration in plasma and serum and in purified test systems. Fibrinogen in high concentrations affects the assay, probably by interfering with the interactions of 125I-a with antibody. However, at the plasma dilutions used in the assay, there is no significant fibrinogen effect. With this assay, the a-chain concentration in normal plasma is approximately 15 micrograms/ml. This compares with 14 micrograms/ml b-chain in plasma and indicates that all of the a- and b-chains in plasma probably circulate in the form of an equimolar zymogen complex. The serum concentration of a-protein is about 6% of the plasma concentration. There is a high correlation between a-protein and factor XIII activity.
Premature chromosome condensation studies in human leukemia: 4. Characterization of albumin density fractionations of bone marrow at presentation, remission, and relapse
Authors: Hittelman WN , Vellekoop L , Zander AR , Dicke KA 
Published: October 2019

Abstract
Previous studies have suggested that the technique of premature chromosome condensation (PCC) is useful in the study of human leukemia, both as a predictive tool for course of disease and as a probe to better understand the biology of the disease process. The purpose of this study was to determine how the various subcomponents of bone marrow populations contribute to the overall PCC pattern. Thirty bone marrow aspirations from persons at various stages of disease (2 normal, 11 untreated, 12 in remission, and 5 n relapse) were fractionated according to density in albumin gradients, and the various fractions were characterized by determining the proliferative potential index (PPI, or the fraction of G1 cells in late G1) using the PCC technique. In general, normal bone marrow and marrow from patients In remission showed lower PPI values throughout the gradients, with the most dense and most mature cells yielding the lowest PPI values in the gradient. In contrast, bone marrow from newly presenting patients and from patients in relapse showed higher PPI values, with the highest PPI values found In the most dense fractions containing the most mature cells. Thus, residual mature cells from patients with active disease exhibit the malignant characteristic of accumulating in late G1 phase. It is postulated that these morphologically mature cells from patients with active disease either represent matured cells of malignant origin or represent residual normal cells pushed beyond the restriction point in early G1 phase by a putative tumor growth factor synthesized by the leukemic cell population.
Characterization of the IgG-Fc receptor on human platelets
Authors: Karas SP , Rosse WF , Kurlander RJ 
Published: October 2019

Abstract
To determine quantitatively the number and avidity of receptors for the Fc portion of IgG on human platelets, we have measured the binding to platelets of human monomeric monoclonal IgG, and of small covalently crosslinked polymers of IgG1 labeled with 125I. The binding of labeled IgG1 monomers to platelets is too weak to permit quantitation. The binding of dimers or larger polymers of IgG1 is much more avid (greater at 4 degrees C than 37 degrees C), is readily reversible, and is saturable. The number of receptor sites ranges from 400 to 2000 per platelet and the mean equilibrium association constant (Ka) for the binding of dimers at 4 degrees C is 2.2 x 10(7) M-1 +/- 0.9 x 10(7) M- 1. The binding is specific for the Fc portion of IgG, and IgG1 and IgG3 bind to the receptors much more avidly than IgG2 or IgG4. Unlabeled IgG1 dimers are about 7--8-fold more potent in inhibiting binding than are IgG1 monomers, and larger polymers are even more potent than dimers. Thus, the Fc receptors on platelets bind human IgG1 with the same specificity and similar avidity as Fc receptors on polymorphonuclear leukocytes (PMNs), but PMNs have about 300-fold more receptors per unit of surface area than platelets.
An alternative extrinsic pathway of human blood coagulation
Authors: Marlar RA , Kleiss AJ , Griffin JH 
Published: October 2019

Abstract
To study the interrelationships of the major human coagulation pathways, factor X activation in normal and various deficient human plasmas was evaluated when clotting was triggered by dilute rabbit or human thromboplastin. Various dilutions of thromboplastin were added to plasma samples containing 3H-labeled factor X, and the time course of factor X activation was determined. At a 1/250 dilution of rabbit brain thromboplastin the rate of factor X activation in factor VIII or factor IX deficient plasma was only 10% of the activation rate seen for normal or factor XI deficient plasma. Reconstitution of the deficient plasmas with factors VIII or IX, respectively, restored normal factor X activation. Similar results were obtained when various dilutions of human thromboplastin replaced the rabbit thromboplastin. From these experiments, it is inferred that normal activation of factor X in plasma due to dilute thromboplastin requires factors VII, IX and VIII. An alternative extrinsic pathway that involves factors VII, IX, and VIII may be a major physiologic extrinsic pathway, and this pathway may help to explain the clinical observations of bleeding diatheses in patients deficient in factors IX or VIII.
Deficiency of methylthioadenosine phosphorylase in human leukemic cells in vivo
Authors: Kamatani N , Yu AL , Carson DA 
Published: October 2019

Abstract
Cells from 20 patients with leukemia and 9 with solid tumors were assayed for the enzyme methylthioadenosine phosphorylase, which function in both purine and polyamine metabolism in rapidly dividing cells. As determined by autoradiography of viable cells, and by direct enzymatic analysis, samples from one individual with pre-T-cell acute lymphoblastic leukemia and one with common acute lymphoblastic leukemia were methylthioadenosine phosphorylase deficient. In contrast, other leukemias of similar antigenic phenotype, as well as normal peripheral blood lymphocytes, thymic lymphocytes, and normal bone marrow cells, had substantial methylthioadenosine phosphorylase activity. This evidence suggests that the complete absence of methylthioadenosine phosphorylase distinguishes some leukemic cells in vivo from their nonmalignant counterparts.
Detection of hemophilia carriers during pregnancy
Authors: Hoyer LW , Carta CA , Mahoney MJ 
Published: October 2019

Abstract
The accuracy of hemophilia A carrier detection during pregnancy has been determined using combined measurement of VIII:CAg and VIIIR:Ag. These immunoassays detect determinants that are sufficiently stable in plasma that the assays could be done on frozen samples that had been obtained when women were seen for antenatal diagnosis studies (carrier women) or for routine prenatal care (controls). A linear discriminant was calculated that best separated the data for 32 normal women and 25 obligate carriers of the hemophilia gene. Twenty-three of 25 carriers (92%) and all 32 control women were correctly identified in this analysis. The overall classification accuracy (55/57, 96%) is comparable to that obtained by VIII:C and VIIIR:Ag measurements using freshly drawn blood samples in nonpregnant individuals. This study demonstrates that hemophilia A carriers can be detected during pregnancy with sufficient accuracy that the information may be used for genetic counseling.
Variant von Willebrand's disease type B--revisited
Authors: Howard MA , Salem HH , Thomas KB , Hau L , Perkin J , Coghlan M , Firkin BG 
Published: October 2019

Abstract
Results of investigations of the factor VIII (FVIII) of a patient with an unusual variant form of von Willebrand's disease (vWD) are presented. A two-peak crossed-immunoelectrophoresis (CIE) pattern was seen when fresh plasma was electrophoresed, but the CIE pattern became normal by incubating the plasma at 37 degree C for more than 72 hr. The two peaks on CIE were separated by cryoprecipitation: the slow-moving peak precipitating and the fast-moving forms of FVIII remaining in the cryosupernate. An additional protein band was seen on multimeric analysis of FVIII. The platelet-rich plasma (PRP) from this patient did not respond to ristocetin, but agglutinated normally in response to botrocetin. Multimeric and CIE analysis of the FVIII post agglutination and 125I-FVIII binding studies to normal formalin-fixed platelets indicated that this patient's FVIII interacted normally with botrocetin but failed to interact with ristocetin. These data strongly suggest that the sites on the FVIII molecule or the multimeric forms involved for ristocetin and botrocetin are different and that the ristocetin reaction is more closely aligned to the physiologic function of FVIII.
Induction of differentiation of human myeloid leukemias: surface changes probed with monoclonal antibodies
Authors: Ferrero D , Pessano S , Pagliardi GL , Rovera G 
Published: October 2019

Abstract
The surface changes occurring in three acute myeloid leukemia cell lines (HL60, ML3, and KG1) induced to differentiate by a variety of agents (dimethylsulfoxide, retinoic acid, 12-O-tetradecanoylphorbol-13- acetate, and factors present in lymphocyte conditioned medium) were probed using monoclonal antibodies that are differentiation stage- and lineage-specific. In all cases, the differentiated phenotype was defective and varied with the inducing agent and the cell line used. HL60 proved to be the most sensitive to the effect of the inducers. Retinoic acid was better than DMSO, and TPA was better than the medium factors in the ability to induce granulocytic and monocytic differentiation, respectively, in HL60 cells. These findings indicate that the differentiation block in acute myeloid leukemias is heterogeneous and that each cell line has different phenotypic characteristics that are responsible for the extent of differentiation obtained with a given inducer. These results also suggest that the extent of the differentiation response in vitro may be improved by the use of more suitable inducers for each specific leukemic line.
Phenotypic and functional analysis of leukemic cells from 16 patients with adult T-cell leukemia/lymphoma
Authors: Yamada Y 
Published: October 2019

Abstract
Surface phenotypes of leukemic cells from 16 patients with adult T-cell leukemia/lymphoma (ATLL) were analyzed by using monoclonal antibodies (anti-Leu-1, anti-Leu-2a, anti-Leu-3a, anti-HLA-DR and MAS 036 c), and the effect of leukemic cells on PWM-induced normal B-cell differentiation was also studied. The majority of ATLL cells bear Leu-1 and Leu-3a antigen on cell surface but lack Leu-2a antigen and were unreactive with MAS 036 c. These results indicate that ATLL cells are of peripheral inducer/helper T-cell origin. However, contrary to the surface phenotype, ATLL cells from 10 patients showed potent suppressor activity on PWM-induced normal B-cell differentiation to immunoglobulin- producing cells (Ig-PC) and no case showed helper activity. The dissociation between surface phenotype and function of ATLL cells is discussed in this article.