bluuebunny/crossref_metadata_2025_split huggingface dataset:
Abnormal platelet function and arachidonate metabolism in chronic idiopathic thrombocytopenic purpura
Authors: Stuart MJ , Kelton JG , Allen JB 
Published: October 2019

Abstract
We observed several patients with chronic idiopathic thrombocytopenic purpura (ITP) whose bleeding times were more prolonged than would have been expected from their platelet counts. To investigate this further, we performed in vivo and in vitro platelet function studies, assessed arachidonate metabolism, and measured platelet-associated IgG (PAIGG) in seven patients with chronic ITP. The bleeding times of three of the patients were prolonged for greater than 7 min, and all of these patients had impaired platelet aggregation and abnormal platelet arachidonic acid metabolism as reflected by increased production of the lipoxygenase product HETE and a concomitant decrease in cyclooxygenase products, TXB2 and HHT (p less than 0.001). The abnormalities noted were not due to concomitant drug ingestion, since they were present on repeated evaluation. There was no relationship between the platelet count and the bleeding time; however, there was a significant inverse correlation between the bleeding time and TXB2 production in all patients evaluated (r = 0.81; p less than 0.05). There was no relationship between the level of platelet-associated IgG and any parameter of platelet aggregation or arachidonate metabolism. The abnormalities noted should be looked for in the individual patient with chronic ITP, since the bleeding tendency is exacerbated by the superimposed impairment of platelet function even at platelet counts of greater than 50,000/cu mm, levels generally regarded as “safe”.
Effect of multimeric structure of the factor VIII/von Willebrand factor protein on binding to platelets
Authors: Gralnick HR , Williams SB , Morisato DK 
Published: October 2019

Abstract
The characteristics of the intact factor VIII/von Willebrand factor protein binding to human platelets was compared to 2-mercaptoethanol- treated factor VIII/von Willebrand factor protein and to fractions of plasma factor VIII/von Willebrand factor protein that elute after the void volume. These studies indicate that the factor VIII/von Willebrand factor protein larger size oligomers bind preferentially with high affinity to low capacity sites on human platelets. The intermediate and smaller size oligomers bind with intermediate or low affinity to sites with a much greater capacity. The results from binding analysis are also paralleled by the competitive inhibition of the intact factor VIII/von Willebrand factor protein by the various 2-mercaptoethanol- treated materials. These studies indicate that the two classes of binding sites seen in previous reports of factor VII/von Willebrand factor binding reflect heterogeneity in the oligomer size of the factor VIII/von Willebrand factor protein used in these assays. This study provides a model for understanding some of the normal structure- function relationships of the normal factor VIII/von Willebrand factor protein and the defect(s) in a variant form of von Willebrand's disease. In this form of the disease, decreased factor VIII/von Willebrand factor binding to platelets is reflected in decreased von Willebrand factor activity but coagulant and/or antigen levels are normal or only slightly decreased.
Abnormal phosphoenolpyruvate transport in erythrocytes of hereditary spherocytosis
Authors: Ideguchi H , Hamasaki N , Ikehara Y 
Published: October 2019

Abstract
The rate of phosphoenolpyruvate transport in erythrocytes from patients with hereditary spherocytosis and from healthy individuals was examined in a 0.1 M citrate buffer (pH 6.1 at 37 degrees C) containing 10 mM phosphoenolpyruvate and 10 mM NaF. The rate in erythrocytes from patients with hereditary spherocytosis was 0.09 +/- 0.02 mumol/min/ml of cells (mean +/- SD, n = 8), whereas the rate in erythrocytes from healthy individuals was 0.23 +/k- 0.03 mumol/min/ml of cells (mean +/- SD, n = 8). The lower rate of transport in erythrocytes of hereditary spherocytosis could not be described to a different glycolytic response to NaF or to a difference in cell age distribution. This phenomenon seemed to be a reflection of some abnormality in the erythrocyte membrane from patients with hereditary spherocytosis.
Preaggregation reactions of platelets
Authors: Gear AR 
Published: October 2019

Abstract
Whether platelet volume increases during the morphological changes preceding aggregation has been investigated. Previous research is controversial; resistive-counting techniques reveal an increase, centrifugal methods do not. Platelets were sized with a computerized, resistive-particle counter before and after incubation with adenosine diphosphate (ADP). Resistive volume increased by 14% (p less than 0.001) in the absence of EDTA, and only 7% in its presence (ADP, 10 micro M). EDTA inhibited platelet volume changes, whether these were shrinking induced by warming or swelling by ADP. Handling of platelets, such as during centrifugation, also caused particle swelling. Particle density decreased after ADP exposure, without release of serotonin, suggesting uptake of water. Platelet shape was experimentally manipulated to test the hypothesis that resistive volume changes stem from artifacts of particle shape. Scanning electron microscopy confirmed that colchicine, chlorpromazine, and a temperature cycle of 0 degrees to 37 degrees all caused extensive alteration from the disc shape. Subsequent exposure to ADP increased resistive volume, and in the case of chlorpromazine, no long pseudopodia were extruded. It is concluded that preaggregation reactions of platelets can be associated with an increase in particle volume, and that earlier research based on centrifugation and the presence of ETA failed to reveal the increase because of inhibitory and apparent swelling effects.
Monoclonal antibodies to von Willebrand's factor: reactivity with porcine and human antigens
Authors: Katzmann JA , Mujwid DK , Miller RS , Fass DN 
Published: October 2019

Abstract
Monoclonal antibodies to porcine von Willebrand's factor (vWf) were obtained from hybridomas derived from the fusion of NS-1 plasmacytoma cells and spleen cells from immunized BALB/c mice. Twenty hybrids were positive in a radioimmunoassay based on the ability of immobilized hybridoma antibodies to bind porcine vWf as detected by the subsequent binding of 125I-polyclonal rabbit anti-vWf. Growth of the hybridomas as ascitic tumors yielded fluids having titers in this assay ranging from 8 x 10(7) to 8 x 10(11). All the antibodies were positive when normal, but not von Willebrand's, plasma was used as the source fo antigen in this RIA, and when normal, but not von Willebrand's, acetone-fixed platelets were used in an indirect immunofluorescence assay. Two of these antibodies inhibited both ristocetin-induced platelet aggregation and platelet aggregating factor. In a second radioimmunoassay, only 13 were of the 20 antibodies were reactive with human vWf, and two of these would not react with human antigen in the presence of 2M Nacl. Bases on these assays, six reactivity groups of antibodies could be identified.
A human b-cell lymphoma synthesizing and expressing surface mu-chain in the absence of detectable light chain
Authors: Gordon J , Hamblin TJ , Smith JL , Stevenson FK , Stevenson GT 
Published: October 2019

Abstract
Lymph node cells obtained from a case of non-Hodgkin's lymphoma, of follicular center cell histology, were seen by immunofluorescence to express immunoglobulin mu heavy chains but no detectable light chain on their surfaces. Antibody specific for determinants expressed only on uncombined mu-chain reacted with these cell surfaces, but not with normal or neoplastic cells expressing surface IgM. Analysis of radioiodinated surface material revealed mu-chains which, without reduction, migrated on SDS-gel electrophoresis as normal mu-chain monomers, and again failed to detect light chains. Lysates of cells incubated with 3H-leucine revealed the synthesis of mu-chains but not of light chains. The labeled mu-chains were not secreted into the culture supernatant, a finding confirmed in parallel cultures by radioimmunoassay. The findings are discussed with particular reference to current concepts of the “mu-chain only” phenotype.
The sickle gene polymorphism in North Africa
Authors: Mears JG , Beldjord C , Benabadji M , Belghiti Ya , Baddou MA , Labie D , Nagel RL 
Published: October 2019

Abstract
Analysis of the restriction endonuclease Hpa 1-beta globin gene linkage has been performed in a predominantly Arab population of North Africa possessing the sickle (beta A) gene is found associated with a 7.6 kilobase) or 7.0 kb Hpa 1 fragment (54/54 assignable beta A genes), whereas the beta S gene is found associated with a 13 kb Hpa 1 fragment (42/42 assignable beta S genes). The results demonstrate a very tight linkage of the beta S gene to the 13 kb Hpa 1 fragment as well as a very low probability that a beta A gene will be found on a 13 kb Hpa 1 fragment. Thus, the North African population presents a nearly ideal opportunity for prenatal diagnosis solely by Hpa 1-beta globin gene linkage analysis. Additionally, the evidence supports the hypothesis that the beta S gene flowed from West Africa rather than from Arab populations in the Middle East.
Normal pregnancy in a patient with a prior postpartum factor VIII inhibitor: with observations on pathogenesis and prognosis
Authors: Coller BS , Hultin MB , Hoyer LW , Miller F , Dobbs JV , Dosik MH , Berger ER 
Published: October 2019

Abstract
A 22-yr-old primigravada developed a hemorrhagic diathesis 6 days after delivering a normal female infant and was found to have an immunoglobulin inhibitor of factor VIII (15 Bethesda units). The patient was treated with prednisone and the bleeding stopped soon thereafter. Her inhibitor titer decreased over the next 8 mo, at which time no inhibitor was detectable. Nine months later she became pregnant again and proceeded to have an uneventful pregnancy and delivery of a normal female infant without evidence of a recurrence of the inhibitor. Studies of 3H-thymidine incorporation into the patient's lymphocytes in the presence of her own plasma or plasmas from her husband, normals, a von Willebrand patient, and a hemophilic patient yielded equivocal results. Analysis of VIII:CAg using 2 different antisera failed to discern immunologic differences between the VIII:CAg of the patient, her husband, or her first child. A review of the literature revealed that there were no recurrences with second pregnancies in any of the 8 patients with postpartum factor VIII inhibitors whose inhibitors had completely disappeared prior to delivery. While the pathogenesis of this disorder remains uncertain, the apparently favorable prognosis for such patients should be considered in counselling with regard to future pregnancies.
Labeled factor IX kinetics in patients with hemophilia-B
Authors: Smith KJ , Thompson AR 
Published: October 2019

Abstract
Labeled factor IX was infused five time into four patients with hemophilia-B. Ten-minute plasma recovery average 35% (SD +/- 2) and the mean T 1/2 beta-phase elimination was 23 hr (+/- 5). No alteration in the postinfusion 125I-factor-IX could be detected by radioautography of plasma samples run on polyacrylamide gels or on crossed- immunoelectrophoresis. Label was excreted into the urine as free 125I- iodide. Kinetics were similar when the labeled preparation was infused alone or with a commercial concentrate containing unlabeled factor IX. Infusion of factor IX in man is best described by a two-compartment open pharmacokinetic model where factor IX is distributed in a space larger than the plasma volume.
Granulopoietic differentiation in AML blasts in culture
Authors: Marie JP , Izaguirre CA , Civin CI , Mirro J , McCulloch EA 
Published: October 2019

Abstract
A newly described monoclonal antibody detected an antigen (My-1) that is expressed on the cell surface during normal granulopoietic differentiation. The kinetics of expression of My-1 and NASD chloracetate esterase were studied during the formation in culture of blast colonies and normal granulopoietic colonies. My-1-positive cells increased during blast colony formation in 6 of 9 experiments, while NASD esterase-positive cells decreased. In contrast, in normal granulopoiesis in culture both markers increased coherently. We suggest that components of granulopoietic differentiation programs are expressed abnormally during blast colony formation in culture.
Elevated platelet-bound IgG associated with an episode of thrombotic thrombocytopenic purpura
Authors: Sims PJ , Boswell EB 
Published: October 2019

Abstract
The level of platelet-associated IgG (PAIgG) were measured during the successful treatment of a patient with thrombotic thrombocytopenic purpura. Prior to therapy. PAIgG was found to be markedly elevated to 195 fg/cell (normal range 0--3.5 fg/cell). The institution of combined therapy with intensive plasma exchange transfusions, high-dose steroids, and antiplatelet drugs resulted in a complete recovery and a decline in PAIgG to the normal range. The possible role of platelet antibody in the pathogenesis of this disorder is discussed.
Chemotactic deactivation of human neutrophils: protective influence of phenylbutazone
Authors: Nelson RD , Gracyk JM , Fiegel VD , Herron MJ , Chenoweth DE 
Published: October 2019

Abstract
The antiinflammatory drug, phenylbutazone (PBZ), has been studied in terms of its influence on chemotactic deactivation of human neutrophils. When PBZ was present during the time of preincubation of cells with N-formyl-methionyl-phenylalanine (F-Met-Phe), loss of subsequent spontaneous mobility and chemotactic responsivity to F-Met- Phe did not occur. The action of PBZ to protect neutrophils from peptide-mediated chemotactic deactivation was found to involve in part its inhibitory influence on hexose monophosphate shunt activity and in part its antagonistic effect on interaction of peptide receptors with N- formyl peptide. Phenylbutazone interfered with binding of N-formyl- methionyl-leucyl-[3H]phenylalanine but not [125I]C5a to the neutrophil, displaced labeled tripeptide bound in the absence of PBZ, increased the dissociation constant (KD) for labeled tripeptide, and limited down regulation of peptide receptor function. These results provide an example of drug-mediated modulation of the interaction of neutrophils with N-formyl peptide and strongly support the possibility that PBZ interacts directly and specifically with the human neutrophil peptide receptor as a competitive antagonist. They also provide an additional example of a compound outside of th N-formyl peptide series that interacts with the peptide receptor.
An autoantibody reactive with nuclei of polymorphonuclear neutrophils: a cell differentiation marker
Authors: Whittingham S , Morstyn G , Wilson JW , Vadas MA 
Published: October 2019

Abstract
An autoantibody that reacted with nuclei of polymorphonuclear neutrophils (PMN) was detected at titers of greater than 10 in sera of 25 of 50 patients with rheumatoid arthritis and 36 of 50 with autoimmune chronic active hepatitis but in none of 160 controls comprising 24 patients with alcoholic cirrhosis, 36 with multiple myeloma, and 100 healthy subjects. Through the use of enriched populations of hemopoietic cells, this antibody was shown to be cell- specific, reacting only with the nucleus of the mature neutrophil. It was unreactive with nuclei of progenitor cells in the myeloid series and with nuclei of eosinophils, monocytes, lymphocytes, and thymocytes. It reacted with a determinant that appeared to be a differentiation antigen. This cell-specific autoantibody may prove to be of value in analytical studies of granulocyte maturation.
Histochemical demonstration of terminal deoxynucleotidyl transferase in leukemia
Authors: Hecht T , Forman SJ , Winkler US , Santos S , Winkler KJ , Carlson F , Maslow WC , Borer WZ , Blume KG 
Published: October 2019

Abstract
White blood cells from 22 patients with leukemia and lymphoma were studied for the presence of terminal deoxynucleotidyl transferase with a peroxidase-antiperoxidase method. The enzyme was detected in leukemic cells of 5 patients with acute lymphoblastic leukemia and 1 patient with chronic myelogenous leukemia, whereas 16 patients with different forms of leukemia or lymphoma were negative for this enzyme. Comparative studies using a biochemical and an indirect immunofluorescence assay revealed complete concordance between these three methods.
A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations
Authors: Schooley RT , Flaum MA , Gralnick HR , Fauci AS 
Published: October 2019

Abstract
The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.
Hemophilic dog model for evaluating therapeutic effectiveness of plasma protein fractions
Authors: Kingdon HS , Hassell TM 
Published: October 2019

Abstract
Therapeutic effectiveness of factor VIII inhibitor bypassing materials has been evaluated in dogs with hemophilia A. A standardized template gingival biopsy was performed using local anesthesia. Hemophilic dogs bled extensively from the biopsy site, whereas in normal dogs the wound was sealed within 5 + /- 2 min. If untreated, the hemophilic dogs frequently bled for several days. Factor VIII infusion stopped the bleeding promptly. Some experimental preparations of factor VIII inhibitor bypassing materials were shown to be therapeutically effective, whereas others were not. Intravascular thrombi could not be demonstrated histologically. The model should prove useful for evaluating factor VIII inhibitor bypassing materials and also for evaluating their mechanism of action.
Variant von Willebrand's disease and pregnancy
Authors: Hanna W , McCarroll D , McDonald T , Painter P , Tuller J , Chen J , Lange R 
Published: October 2019

Abstract
The clinical course and coagulation profile of a pregnant patient with variant von Willebrand's disease were followed from the second trimester through puerperium. The clinical course was characterized by a normal delivery and absence of abnormal bleeding or need for replacement therapy. The coagulation profile demonstrated an increase in factor VIII procoagulant activity, factor-VIII-related antigen, and platelet aggregation activity in response to ristocetin prior to delivery. Postpartum, these factors decreased to prepregnancy values with distinctly different patterns. Factor VIII procoagulant activity continued to rise for 5 days after delivery and then decreased with a half-life of approximately 6 days. Factor-VIII-related antigen began to decrease just prior to delivery, displaying a half-life or approximately 6 days. Ristocetin cofactor activity, however, dropped immediately postpartum and displayed a half-life of approximately 6 hr. The ristocetin cofactor activity was associated with factor-VIII- related antigen, which displayed a significantly smaller molecular weight than does normal factor-VIII-related antigen. Larger aggregates of factor-VIII-related antigen. Larger aggregates of factor-VIII- related antigen did not appear during the pregnancy, and ristocetin cofactor activity could not be demonstrated in fragments of less than 0,8 x 10(6).
Heterogeneity in human prothrombin: analysis of cause
Authors: Bajaj SP , Rapaport SI , Prodonos C , Russell WA 
Published: October 2019

Abstract
Two fractions of human prothrombin can be isolated from single donor plasma by the technique of heparin-agarose chromatography in (sodium) citrate buffer, pH 7.5, as previously reported for pooled plasma. The two fractions, designated H-II1 and H-II2, are found in a ratio of approximately 4:1. Both forms comigrate in sodium dodecyl sulfate gel electrophoresis; however, under nondenaturing electrophoretic conditions, each fraction migrates as a discrete entity with a different mobility. The larger fraction (H-II1) has a faster mobility towards the anode. Isoelectric focusing in urea of H-II1 reveals that it has two components, a minor component with a pl of 5.25 (H-II1a) and a major component with a pl of 5.40 (H-II1b). H-II2 has a pl of 5.6 H- II1 and H-II2 possess the same amino terminal residue (alanine, 0.87- 0.92 mole/mole) and the same number of gamma -carboxyglutamic acid residues (9.8–10.5). Their amino acid composition is indistinguishable. However, the two fractions of prothrombin differ in their content of neutral sugar and of sialic acid residues. Removal of sialic acid with neuraminidase abolishes the electrophoretic heterogeneity. Thus, the charge heterogneity of the three variants of prothrombin found in normal human plasma appears to result exclusively from differences in the number of sialic acid residues attached to the protein moiety of the molecule.
Immunologic abnormalities in myelofibrosis with activation of the complement system
Authors: Gordon BR , Coleman M , Kohen P , Day NK 
Published: October 2019

Abstract
Eighteen patients with agnogenic myeloid metaplasia with myelofibrosis were studied for clinical and laboratory evidence of immunologic dysfunction. Clinical findings included the presence of arthritis, vasculitis, and erythema nodosum. Laboratory abnormalities included the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs tests, elevated latex fixations, and a circulating lupus type anticoagulant. Total hemolytic complement was markedly depressed in four patients. Analysis of complement (C) components C1-C9 and factor B demonstrated significant reduction of only C3 and factor B. By crossed-immunoelectrophoresis, both C3 and factor B, but not C4, were cleaved, indicating that C activation was occurring predominantly via the alternative pathway. The control proteins beta 1H and C3b inactivator were decreased in three of four patients with hypocomplementemia. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis.