bluuebunny/crossref_metadata_2025_split huggingface dataset:
Inheritance of porcine von Willbrand's disease: study of a kindred of over 700 pigs
Authors: Fass DN , Bowie EJ , Owen CA Jr , Zollman PE 
Published: October 2019

Abstract
A study of over 700 pigs affected with von Willebrand's disease suggests that the classic disease is transmitted as an autosomal recessive trait. Less clear is the genetic basis of inheritance of variant types of this disease produced by selective mating.
Treatment of refractory splenomegaly in myeloproliferative disease by splenic artery infusion
Authors: Canellos GP , Sutliffe SB , DeVita VT , Lister TA 
Published: October 2019

Abstract
Five patients in the blastic phase of chronic granulocytic leukemia with massive splenomegaly were treated by intraarterial splenic artery infusion of cytosine arabinoside. All patients had massive splenomegaly associated with pain and/or hypersplenism and were refractory to previous therapy. All 5 patients demonstrated responses to treatment, with reduction in spleen size as well as symptomatic relief. Systemic toxicity was minimal in 4 of the 5 patients.
Two populations of granulocytes in paroxysmal nocturnal hemoglobinuria
Authors: Stern M , Rosse WF 
Published: October 2019

Abstract
The granulocytes in paroxysmal nocturnal hemoglobinuria (PNH) are defective, and the defect is similar to that previously described for the PNH erythrocyte. Using anti-I antibody to activate complement and 51Cr release to detect cell lysis, we found two populations of granulocytes that differed in their susceptibility to lysis by complement in 5 of 6 patients. A proportion of the cells were lysed by one-fifteenth to one-twentieth the amount of complement required to lyse normal cells; the remainder of the granulocytes appeared to be normal in their susceptibility to the lytic action of complement. The binding of the third component of complement (C3) to PNH granulocytes was at least twice that bound to normal cells, even though the binding of antibody was the same for normal and PNH cells. This suggests that the binding of C3 and probably the efficiency of the terminal steps of complement lysis are increased in the abnormal PHN granulocyte. These defects affect only a portion of the granulocytes, thus suggesting that the disorder is a clonal stem cell abnormality.
T-cell-subset characterization of human T-CLL
Authors: Reinherz EL , Nadler LM , Rosenthal DS , Moloney WC , Schlossman SF 
Published: October 2019

Abstract
Circulating peripheral blood tumor cells in four cases of chronic lymphoproliferative disease were immunologically characterized. By the use of T-cell-specific heteroantisera and indirect immunofluorescence, all were shown to involve proliferation of malignant T cells. Three cases demonstrated morphologic and clinical features consistent with chronic lymphocytic leukemia (CLL), and one case presented as a lymphosarcoma cell leukemia. Antisera specific for normal human T-cell subsets defined the malignant T cells in each case as arising from the TH2--subset. This subset normally constitutes approximately 80% of human peripheral blood T cells. Terminal deoxynucleotidyl transferase (TdT) was not detected in any of the T-cell CLL cases, thus supporting the notion that T-cell CLL represents a malignancy of a mature phenotype. The one patient with lymphosarcoma whose tumor cells were TdT-positive subsequently developed T-cell acute lymphoblastic leukemia (ALL). Moreover, la-like antigen (p23,30) was detected on two of these tumor cell populations. In addition, it was shown that not all tumor cells were E-rosette-positive, since only cells from 3 of 4 patients were capable of forming spontaneous rosettes. These findings demonstrate that heteroantisera can provide an additional important tool for dissecting the heterogeneity of T-cell leukemias and for relating them to more differentiated normal T cells.
Fractionation of antibodies to L-cell colony-stimulating factor by affinity chromatography
Authors: Shadduck RK , Waheed A , Pigoli G , Boegel F , Higgins L 
Published: October 2019

Abstract
Purified L-cell colony-stimulating factor (CSF) was coupled to cyanogen- bromide-activated Sepharose and used to selectively fractionate antibodies to this factor. With the use of a simplified two-step washing and elution technique, there was 50%--70% binding of the anti- CSF, with recovery of 60%--100% of the bound material. Both the native antiserum and purified anti-CSF fractions were inhibitory to murine granulocyte-macrophage colony formation. The purified antibodies contained only IgG and were reduced in protein concentration to 0.1% of the serum IgG values. These fractions should prove useful tools for the study of granulocyte and macrophage differentiation.
Separation of erythroid progenitor cells in mouse bone marrow by isokinetic-gradient sedimentation
Authors: Misiti J , Spivak JL 
Published: October 2019

Abstract
Isokinetic-gradient sedimentation employing a shallow linear gradient of Ficoll in tissue culture medium was used to isolate erythroid progenitor cells (CFU-e) from mouse bone marrow. Following gradient sedimentation, 34% of the total nucleated cells and 48% of the CFU-e applied to the gradient were recovered, and three distinct modal populations of CFU-e could be distinguished. The slowest-migrating population did not require exposure to exogenous erythropoietin in order to form erythroid colonies in vitro. The other two modal populations of CFU-e required exposure to exogenous erythropoietin for differentiation. One of these, constituting 64% of the hormone- dependent CFU-e recovered, migrated with the bulk of the marrow cells, whereas the other migrated ahead of the bulk of the marrow cells. This latter population, which contained 34% of the CFU-e, was recovered with 11% of the marrow cells, representing a twofold to threefold enrichment. BFU-e migrated more slowly than the erythropoietin- dependent CFU-e. Resedimentation studies suggested that the two erythropoietin-dependent CFU-e populations were distinct modal populations. When cells from the fastest-migrating population of erythropoietin-dependent CFU-e were cocultured with unseparated marrow cells, a further twofold to threefold enhancement of erythroid colony formation was obtained. Comparison of isokinetic-gradient sedimentation with discontinuous and continuous albumin density-gradient sedimentation revealed that isokinetic-gradient sedimentation was a more efficient method than the former and a more rapid method than the latter for isolating CFU-e from mouse bone marrow.
A phosphorus-31 nuclear magnetic resonance investigation of intracellular environment in human normal and sickle cell blood
Authors: Lam YF , Lin AK , Ho C 
Published: October 2019

Abstract
Intracellular pH and 2,3-diphosphoglycerate concentration in sickle cell amenia and normal human blood samples were measured by means of phosphorus-31 nuclear magnetic resonance spectroscopy. To monitor the concentrations of various internal phosphorylated metabolites of intact red blood cells, heparinized blood samples were used and were incubated at 37 degrees C with 5.6% C92, 25% O2, and 69.4% N2. The 31P chemical shifts of phosphorylated compounds, such as 2,3-diphosphoglycerate, adenosine 5′-triphosp-ate, and inorganic phosphate, depend on pH, and by using an appropriate calibration curve, the intracellular pH of intact erythrocytes can be obtained. The intracellular pH values in fresh sickl cell blood and normal blood were found to be 7.14 and 7.29, respectively. However, the whole-blood pH, as measured by a standard pH meter, was found to be 7.54 for both types of blood. The initial concentration of 2,3-diphosphoglycerate in sickle cell blood was about 30% higher, but it was depleted much faster during incubation than that in normal blood. The difference in intracellular pH between these two types of blood samples remained constant during incubation, even after depletion of 2,3-diphosphoglycerate. These results suggest that there are differences in intracellular environment between normal and sickle cell blood. Thus, 31P nuclear magnetic resonance spectroscopy provides a fast, direct, continuous, and noninvasive way to monitor the intracellular environment of intact erythrocytes.
Comparison of techniques for detecting T-cell acute lymphocytic leukemia
Authors: Melvin SL 
Published: October 2019

Abstract
Three versions of the E-rosette test, one using untreated sheep erythrocytes at 37 degrees C, another using such cells at 4 degrees C, and a third using sheep erythrocytes treated with S-(2- aminoethyl)isothiouronium bromide hydrobromide (AET), were applied to each of 72 bone marrow specimens from as many unselected patients with untreated acute lymphocytic leukemia (ALL). The same specimens were also examined for T-cell antigens, based on reactivity with an antithymocyte serum. Lymphoblasts in eight ALL specimens formed E rosettes at 37 degrees C; no other E-positive specimens were identified when the assay was done at 4 degrees C. With AET-treated erythrocytes, lymphoblasts from these eight specimens and six additional specimens readily formed rosettes. T-cell antigens were detectable in all specimens positive for rosette formation withe untreated erythrocytes, in four of the six specimens positive for rosette formation with AET- treated erythrocytes, and in four specimens that showed no rosette formation under any of the experimental conditions used. Altogether, 18 specimens contained lymphoblasts with one or more surface markers characteristic of T-cell leukemia. These findings indicate that more specimens are likely to be identified at T-cell luekemias when E- rosette tests of increasing sensitivity and assayss for T-cell antigens are used. Some leukemic blasts do not possess the full array of membrane receptors and antigens usually associated with T cells. A combination of E-rosette tests and serologic tests is necessary to determine reliably the relationship of the test specimen to either T- cell ALL or common ALL and to establish the clinical significance of blasts that express membrane properties intermediate between those of T- cell ALL and common ALL.
In vitro cytotoxic response to human myeloma plasma cells by peripheral blood leukocytes from patients with multiple myeloma and benign monoclonal gammopathy
Authors: Paglieroni T , MacKenzie MR 
Published: October 2019

Abstract
Peripheral blood leukocytes (PBL) from myeloma patients were studied for their capacity to lyse plasma cells from myeloma patients, benign monoclonal gammopathy (BMG) patients, and nonneoplastic disease patients. Plasma cells were isolated from bone marrow, labeled with 51Cr, and cultured with PBL isolated from patients with myeloma, BMG, or nonneoplastic disease, as well as normal individuals. PBL from patients with multiple myeloma demonstrated responses to autologous or allogeneic myeloma plasma cells. Optimum conditions for cytotoxic response included a responder-to-stimulator ratio of 1:1 and an effector-to-target ratio of 20:1. PBL from normal individuals or patients with BMG failed to demonstrate this response. However, PBL from BMG patients, but not normal individuals, could be induced to kill myeloma plasma cells (but not nonmyeloma plasma cells) by simultaneous stimulation with allogeneic lymphocytes and myeloma plasma cells.
Characterization of lymphoblast Fc receptor expression in acute lymphoblastic leukemia
Authors: Reaman GH , Pichler WJ , Broder S , Poplack DG 
Published: October 2019

Abstract
Lymphoblasts from 18 patients with untreated acute lymphoblastic leukemia were investigated for the presence of conventional cell surface markers (spontaneous sheep red blood cell rosette formation, complement receptors, and surface immunoglobulin). The expression of Fc receptors for both IgG and IgM was investigated using indicator bovine erythrocytes coated with rabbit anti-BRBC (IgG and LgM fractions). The leukemic cells of all patients in this tudy expressed Fc receptors for both IgC and LgM. In contrast with previous reports, null (non-T non-B) lymphoblasts as well as T lymphoblasts demonstrated Fc-IgG and Fc-IgM receptors. However, these two immunologic subclasses of leukemic cells demonstrated significantly different patterns of Fc receptor distribution. These data suggest that expression of Fc receptors is an early event in cellular differentiation in this lymphoid malignancy.
Tn polyagglutination preceding acute leukemia
Authors: Ness PM , Garratty G , Morel PA , Perkins HA 
Published: October 2019

Abstract
Tn polyagglutination (persistent mixed-field polyagglutination) was detected in the blood of a 66-yr-old male laborer at the time of a splenectomy for life-threatening thrombocytopenia. Confirmation that the polyagglutination was caused by Tn activation was established by the use of lectins, by failure of the patient's red cells to react with sera from other patients with Tn polyagglutination, by weak aggregation with polybrene, by low red cell sialic acid levels, and by the persistence of polyagglutination over several years of testing. Two years after the discovery of the Tn polyagglutination, the patient developed acute myelomonocytic leukemia. Vigorous chemotherapy regimens resulted in clinical remission of the leukemia and the Tn polyagglutination. This report describes the first known case of Tn polyagglutination preceding the development of acute myelogenous leukemia.
The effectiveness of rubidazone in hairy cell leukemia (leukemic reticuloendotheliosis)
Authors: Stewart DJ , Benjamin RS , McCredie KB , Murphy S , Keating M 
Published: October 2019

Abstract
Two patients with hairy cell leukemia treated with the anthracycline antibiotic rubidazone are presented. One achieved a complete remission and the other a good partial hematologic and bone marrow remission. Neither has relapsed (at 20 and 13 mo, respectively), and neither has been retreated. Intensive supportive measures were required during the prolonged myelosuppression that followed treatment. The relative youth of the patients (ages 24 and 39 yr) may have contributed to their ability to survive until normal marrow recovered. Chemotherapy should not be employed in the initial management of hairy cell leukemia. However, if life-threatening granulocytopenia and thrombocytopenia occur secondary to bone marrow replacement by leukemic cells, and improvement does not occur using alternative methods of therapy, consideration could be given to chemotherapy with rubidazone. Facilities for intensive supportive care should be available.
Paroxysmal nocturnal hemoglobinuria associated with infectious mononucleosis
Authors: Vogel SJ , Reinhard EH 
Published: October 2019

Abstract
A previously healthy 16-yr-old girl was found to have pancytopenia, low reticulocyte count, a cellular bone marrow, and a negative Coombs test, all coincident with clinical and laboratory evidence of infectious mononucleosis. Symptoms and signs of infectious mononucleosis subsided, but pancytopenia and hemolytic anemia persisted. Sucrose hemolysis and acid hemolysis tests supported a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). After 18 mo, the platelet count is normal, but leukopenia and hemolytic anemia continue. The development of PNH in this patient suggests it may have resulted from an effect of infectious mononucleosis.
Decrease of the major high molecular weight surface glycoprotein of human granulocytes in monosomy-7 associated with defective chemotaxis
Authors: Gahmberg CG , Andersson LC , Ruutu P , Timonen TT , Hanninen A , Vuopio P , de la Chapelle A 
Published: October 2019

Abstract
By use of the galactose/NaB3H4 surface labeling technique followed by polyacrylamide slab gel electrophoresis, it is shown that the major labeled surface glycoprotein (GP130) of normal human blood granulocytes is markedly reduced in granulocytes from three patients with a chromosomal abnormality in all or most bone marrow mitoses. The abnormality consisted of monosomy-7 in two and deletion of the distal half of the long arm of chromosome-7 in the third. The granulocytes from these patients showed reduced chemotaxis. These results suggest that the expression of GP130, as well as the chemotactic ability of the cells, are at least in part controlled by one or several genes on chromosome-7. The GP130 protein may be involved in normal granulocyte chemotaxis.
Drug-induced agranulocytosis: in vitro evidence for immune suppression of granulopoiesis and a cross-reacting lymphocyte antibody
Authors: Taetle R , Lane TA , Mendelsohn J 
Published: October 2019

Abstract
Two patients with agranulocytosis associated with diphenylhydantoin (DPH) therapy and clinical data suggesting suppression of granulopoiesis were investigated using in vitro culture techniques for committed granulocyte/macrophage precursors. Addition of DPH to cultures containing the patients' sera resulted in significant suppression of colony growth. Extensive studies on the acute serum from one patient revealed the drug-dependent inhibitory activity to be nondialyzable, resistant to chloroform extraction, heat stable, active in the presence of heat-inactivated fetal bovine serum, active against autologous as well as allogeneic cells, and absent from convalescent sera. Drug-dependent bone marrow colony-suppressing activity was removed by absorption on an antiimmunoglobulin-Sepharose column but not by IgG-Sepharose. The serum show non-drug dependent suppression of oxygen consumption by normal polymorphonuclear leukocytes engaged in phagocytosis and also showed evidence of ability to opsonize these cells. When the serum was incubated with mitogen-stimulated lymphocytes, suppression of 3H-thymidine uptake by autologous but not allogeneic cells was noted. Similarly, the serum suppressed short-term 3H-thymidine uptake by autologous but not allogeneic bone marrow. Absorption of the patients' sera with allogeneic polymorphonuclear leukocytes, autologous polymorphonuclear leukocytes, or autologous lymphocytes removed the drug-dependent inhibitory activity, but absorption with allogeneic lymphocytes did not. These data are most consistent with the presence of a noncomplement dependent antibody capable of suppressing granulopoiesis, mediating peripheral destruction of polymorphonuclear leukocytes, and cross-reacting with a lymphocyte antigen of limited population distribution.
T-cell subpopulations in chronic lymphocytic leukemia: abnormalities in distribtuion and in in vitro receptor maturation
Authors: Kay NE , Johnson JD , Stanek R , Douglas SD 
Published: October 2019

Abstract
Purified human thymus-derived (T) lymphocytes were analyzed by detection of Fc receptors for either IgG or IgM in healthy controls and in patients with chronic lymphocytic leukemia (CLL). There was a significant and persistent increase in the numbers of T cells bearing receptors for IgG (Fc gamma) in CLL patients in comparison to the controls. After an in vitro culture period, there was a significantly decreased appearance of cells with IgM receptors (Fcmu) in CLL patients in comparison to the control group. These results indicate an imbalance in circulating T-cell subpopulations for CLL patients. In addition, an in vitro defect in CLL T-cell membrane receptor appearance is present.
The effect of cultured endothelial cells on factor VIII procoagulant activity
Authors: Stead NW , McKee PA 
Published: October 2019

Abstract
Cultured human umbilical vein endothelial cells produce a protein that has von Willebrand factor activity and forms immunoprecipitates with rabbit antibody to purified plasma factor VIII/von Willebrand factor (FVIII/vWF) protein, but it has no FVIII procoagulant activity. Of the three characteristics of plasma FVIII/vWF protein, only FVIII procoagulant activity is readily destroyed by trace proteases. A previous report from this laboratory demonstrated protease activity in culture medium under conditions that had been used by others to show that endothelial cells do not synthesize protein with FVIII procoagulant activity. However, even if cultured endothelial cells are placed in protease-free culture medium, no FVIII procoagulant activity can be detected, despite an increase in the level of protein with vWF activity from 0 to 0.57 microgram/ml by 48 hr. This observation and the lack of protease activity in medium left in contact with the cells for 48 hr led to the hypothesis that proteases exist on the surface of cultured umbilical vein endothelial cells. Protease activity was quantitated by the hydrolysis of p-nitroaniline from the substrate, N- benzoyl-phenylalanyl-valyl-arginyl-p-nitroanilide and by degradation of the procoagulant activity of added purified plasma FVIII/vWF protein. In the absence of endothelial cells, no protease activity was present in protease-free culture medium whether or not it had previously overlaid cultured cells. This medium did not cause cleavage of p- nitroaniline from the tripeptide substrate, and 83% of added FVIII procoagulant activity remained after 48 hr. When the synthetic tripeptide was incubated in contact with cultured endothelial cells, 7.3 +/- 0.8 X 10(-10) moles of p-nitroaniline/hr was released; moreover, only 47% of the added FVIII procoagulant activity remained after 48 hr. Given this rate of destruction, it can be calculated that sufficient protease activity exists on the surface of cultured endothelial cells to degrade the procoagulant activity of approximately 1.6 microgram FVIII/vWF protein/hr. This degradation rate is 45 times the rate of release of FVIII/vWF protein from cultured endothelial cells when assessed by the generation of protein with vWF activity. Hence, the detection of FVIII procoagulant activity, if in fact synthesized by cultured endothelial cells, will be most difficult.
Loss of Rh antigen associated with acquired Rh antibodies and a chromosome translocation in a patient with myeloid metaplasia
Authors: Cooper B , Tishler PV , Atkins L , Breg WR 
Published: October 2019

Abstract
Myeloid metaplasia is a clonal disease of the marrow pluripotent stem cell in which a constant cytogenetic abnormality could result in an altered antigenic characteristic of hematopoietic cells. A 57-yr-old man who had acquired myeloid metaplasia at age 37 was noted to be Rh negative, although blood typing at age 33 was Rh positive. His erythrocytes reacted with anti-c and anti-e, typical for an Rh negative individual. Analysis of 11 metaphase bone marrow cells by G-banding revealed 46 chromosomes with a consistent anomaly in 100% of cells involving chromosomes 1 and 13. The changes were consistent with a reciprocal translocation, with break points at approximately 1p32 and 13q22, although break points at 1p13 and 13q14 were also possible. Since previous cytogenetic data have localized the Rh gene to the short arm of chromosome 1, our data indicate the Rh gene lies within the segment u13 leads to 1p32. Serum of this patient had circulating anti-D and anti-C antibodies. Consequently, this patient lost immunologic tolerance to a “self antigen” after losing the ability to express this antigen.
A novel lymphocyte differentiating factor in serum of patients with mycosis fungoides and Sezary syndrome
Authors: Safai B , Good RA , Twomey JJ , Lewis V , Goldstein G 
Published: October 2019

Abstract
Sera from 13 patients with mycosis fungoides and 2 with Sezary syndrome were tested for activity that induces lymphocyte differentiation. Induction of Thy-1.2 antigen and surface immunoglobulin were used, respectively, to measure T- and B-cell differentiation. The indicator cells were null lymphocytes from the spleens of congenitally athymic nude mice. Normal serum induced some T-cell but no B-cell differentiation. The T-cell-inducing activity was ascribed to thymic hormone and declined with advancing age. A totally different pattern emerged with patient serum. T-cell-inducing activity was significantly more active than in normal serum (p less than 0.001). This activity did not decline with advancing age and was not inhibited by a concentration of ubiquitin, which blocks nonspecific beta-adrenergic induction. B- cell-inducing activity was also present. This novel serum factor (or factors) is a potent inducer of T- and B-lymphocyte differentiation and is associated with neoplastic lymphoproliferation of the T-cell series.