bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hereditary myeloperoxidase deficiency
Authors: Cech P , Papathanassiou A , Boreux G , Roth P , Miescher PA 
Published: October 2019

Abstract
The functional properties of granulocytes in a diabetic patient deficient in myeloperoxidase (MPO) were compared with those of granulocytes in healthy subjects. The granulocytes of this patient had normal phagocytic activity. The microbicidal activity of the granulocytes was partially diminished with regard to Staphylococcus aureus and was almost nil with regard to Candida albicans. Fungicidal activity of normal granulocytes was shown to be impaired during the in vitro artificial hyperglycemic condition. The relationship among diabetes mellitus, MPO deficiency, and serious C. albicans infection was examined. Genetic investigation was carried out in 28 members of the proband's family. In close relatives of the patient, MPO values were found to be diminshed to a greater or lesser degree, thus suggesting variable expressivity of the heterozygote state of MPO deficiency.
Treatment of acute nonlymphocytic leukemia: use of anthracycline- cytosine arabinoside induction therapy and comparison of two maintenance regimens
Authors: Preisler HD , Rustum Y , Henderson ES , Bjornsson S , Creaven PJ , Higby DJ , Freeman A , Gailani S , Naeher C 
Published: October 2019

Abstract
Patients with acute nonlymphocytic leukemia were given remission induction therapy consisting of cytosine arabinoside and an anthracycline. Those patients who experienced complete remission received two courses of consolidation therapy and were randomized to receive maintenance therapy consisting of either daily chemotherapy with reinforcements every 3 mo or reinforcement therapy only every 6 wk. The overall complete remission rate was 66%, with 80% complete remission for previously untreated patients less than 60 yr of age who did not have a prior history of malignancy. Remission durations were the same for patients treated with both maintenance regimens. The major determinant for successful remission induction therapy was patient age, with older patients frequently succumbing to intercurrent infection. Documented leukemic cell resistance to the therapy employed was only rarely encountered. Once remission was achieved, age was no longer a determinant of patient survival, since duration of remission was independent of age. Remission durations were directly related to leukemic cell retention of cytosine arabinoside triphosphate. Hence therapy for acute nonlymphocytic leukemia can be divided into two separate areas: remission induction and remission maintenance.
Hemoglobin biosynthesis in individual bursts in culture: studies of human umbilical cord blood
Authors: Kidoguchi K , Ogawa M , Karam JD , McNeil JS , Fitch MS 
Published: October 2019

Abstract
We cultured human umbilical cord blood erythropoietic precursors in methyl cellulose clonal assay and analyzed the synthetic rates of Hb A and Hb F in individual erythropoietic bursts. Hemoglobin was labeled with 14C-amino acids in culture, separated by slab gel isoelectric focusing techniques, and quantitated by fluorographic methods. Almost all bursts exhibited both Hb A and Hb F in varying ratios. Frequencies of the individual bursts differing in percentage Hb F biosynthesis had normal distributions. Natal erythropoietic precursors appeared to be randomly committed to Hb F synthesis.
Chromosome analysis in two unusual malignant blood disorders presumably induced by benzene
Authors: Van den Berghe H , Louwagie A , Broeckaert-Van Orshoven A , David G , Verwilghen R 
Published: October 2019

Abstract
Two patients with presumably benzene-induced malignant blood disorders with preleukemic phases were cytogenetically monitored through the courses of their diseases. Patient 1, in addition to a familial chromosome translocation [t(3;16)], developed karyotypic abnormalities in 100% of the marrow cells, including two translocation: t(9;16)] and t(4;15). Monosomy of chromosome 7 characterized the cells of patient 2. Cytogenetic monitoring of the patients at various phases of their diseases served as an important indicator of the transformation or progression of the preleukemia into frank leukemia and of the unusual behavior of such leukemic cells.
Effect of anti-P1A1 antibody on human platelets. I. The role of complement
Authors: Cines DB , Schreiber AD 
Published: October 2019

Abstract
We studied the interaction of complement with human platelets. Complement was activated by IgG anti-P1A1 antibody obtained from 3 patients with the post-transfusion purpura syndrome. We used a heparin- plasma buffer system that permits complement activation and also preserves platelet function. With this system complement activation was efficient, and platelet immune alteration was extensive. Anti-P1A1 antibody was effective only in the presence of complement, in which case both platelet lysis and serotonin release (release reaction) in the absence of lysis were observed. Platelet lysis, as assessed by 51Cr loss, required 10-fold more antibody than was necessary to induce platelet aggregation and release of 14C-serotonin. This platelet release reaction required an intact classic complement sequence through C6. The extent of platelet serotonin release parallelled the depletion of C1 and C4 from platelet-rich plasma. Concentrations of antibody insufficient to induce platelet aggregation and serotonin release could still activate C1 and deposit increased C3 on the platelet surface. These studies demonstrated that complement activation by anti-P1A1 antibody can alter human platelets in a nonlytic system. Several phases of complement-mediated human platelet alteration are possible, depending on the concentration of anti-P1A1 antibody.
Congenital deficiency of blood clotting factors II, VII, IX, and X
Authors: Chung KS , Bezeaud A , Goldsmith JC , McMillan CW , Menache D , Roberts HR 
Published: October 2019

Abstract
A patient congenitally deficient in factors II, VII, IX, and X has been further investigated after a follow-up of 15 yr. At birth, these factors, when determined by clotting assays, were undetectable. Following therapy with vitamin K1, the clotting activity of these factors rose but never exceeded 18% of normal. Immunologic assays revealed much higher levels of these factors than did clotting assays, thus suggesting that the vitamin-K-dependent factors were present in abnormal forms. Two-dimensional crossed immunoelectrophoresis showed that at least two forms of prothrombin were present in the patient's plasma. One form was similar to normal prothrombin; the other had the same mobility as acarboxyprothrombin. In addition, the majority of this fast-migrating peak was not adsorbable onto insoluble barium salts. These observations suggested that some molecules of the patient's prothrombin lacked the normal complement of gamma-carboxyglutamic acid residues. This observation was confirmed by a specific assay for gamma- carboxyglutamate. Since malabsorption of vitamin K, warfarin intoxication, and hepatic dysfunction were excluded as causes of this patient's syndrome, this rare congenital abnormality could represent either a defective gamma-carboxylation mechanism within the hepatocyte or faulty vitamin K transport.
Hematin administration to an adult with lead intoxication
Authors: Lamon JM , Frykholm BC , Tschudy DP 
Published: October 2019

Abstract
Lead poisoning and acute intermittent porphyria (AIP) may exhibit similar neurologic manifestations, and they have in common elevated excretion of urinary aminolevulinic acid (ALA). Despite their similarities, the possible pathophysiologic connection between AIP and lead poisoning in not known. Because intravenous hematin administration has produced biochemical improvement in AIP, a hematin trial in lead intoxication was of interest with respect to some of the heme metabolism abnormalities observed in the condition. Significant diminution of urinary ALA and coproporphyrin excretion occurred in association with intravenous hematin administration.
Clonal identification in acute lymphoblastic leukemia
Authors: Goldstone AH , McVerry BA , Janossy G , Walker H 
Published: October 2019

Abstract
In a case of acute lymphoblastic leukemia, two distinct types of leukemia blast cells could be identified throughout the course of the disease. The initially dominant type of blast cell was sensitive to chemotherapy; the other was drug-resistant, gradually becoming dominant as the disease progressed. The cell types could be clearly separated by their morphologic and surface membrane marker characteristics. The same chromosomal constitution was present in both types of blast cells, indicating a common clonal origin. Additional chromosomal abnormalities were present in the later stages of the disease, demonstrating that a distinct subclone had proliferated. This study illustrates that in some cases of acute leukemia, disease relapse is caused by growth of drug- resistant subclones that may be clearly identified by changes in morphology and surface membrane marker characteristics.
Genetics of classic von Willebrand's disease. II. Optimal assignment of the heterozygous genotype (diagnosis) by discriminant analysis
Authors: Miller CH , Graham JB , Goldin LR , Elston RC 
Published: October 2019

Abstract
In classic von Willebrand's disease (vWd), assignment of the heterozygous genotype for genetic studies and diagnosis for clinical purposes (which are not exactly the same) are formidable problems. We have pointed out in the first report in this series that almost 50% of the members of two large kindred who transmitted this disease, and were therefore heterozygous, were scored as normal by the usual tests of hemostasis. This report describes how this large proportion can be significantly reduced by application of discriminant analysis. Using linear discriminants in three variables--coagulation factor VIII (VIII:C), factor-VII-related antigen (VIIIR:Ag), and the ristocetin cofactor related to factor VIII (VIIIR:WF)--we were able to classify as heterozygous more than 80% of the transmitters in the two large kindred. It was of particular interest that the four parents of two related vWd homozygotes could be scored as heterozygous by discriminant analysis even though all their laboratory tests were within the normal ranges.
Contamination of erythropoietin by endotoxin: in vivo and in vitro effects on murine erythropoiesis
Authors: Zuckerman KS , Quesenberry PJ , Levin J , Sullivan R 
Published: October 2019

Abstract
Endotoxin was detected in all erythropoietin preparations tested and was removed from four lots, without loss of erythropoietic activity, by adsorption with limulus amebocyte lysate. Comparison of adsorbed (endotoxin-depleted) and nonadsorbed (endotoxin-containing) erythropoietin preparations demonstrated significant inhibition of CFU- e and BFU-e in vitro by nonadsorbed erythropoietin at concentrations higher than 0.25 U/ml and 2.0 U/ml, respectively. CFU-e and BFU-e were inhibited significantly by readdition in vitro of 10(-5)-10(-3) mug of endotoxin per unit of limulus-adsorbed erythropoietin. Administration of saline or 6 U of nonadsorbed or adsorbed erythropoietin twice a day for 4 days of CF1 mice resulted in reticulocyte counts of 2.1%, 9.9%, and 15.9%, respectively. Nonadsorbed erythropoietin resulted in a 29% decrease in erythropoiesis, a 42% decrease in CFU-e, and a 16% increase in granulopoiesis in the marrow, whereas adsorbed erythropoietin caused a 28% increase in erythropoiesis, no significant change in CFU-e and a 19% decrease in granulopoiesis in the marrow. Both preparations resulted in marked increases in splenic erythropoiesis and granulopoiesis. The effects of adsorbed erythropoietin are similar to those produced following stimulation of hematopoiesis by endogenous erythropoietin. Hemopoietic changes induced by nonadsorbed erythropoietin in vivo and in vitro are affected substantially by contamination of the erythropoietin preparations with endotoxin.
Characteristics of murine megakaryocytic colonies in vitro
Authors: Burstein SA , Adamson JW , Thorning D , Harker LA 
Published: October 2019

Abstract
Characteristics of murine megakaryocytic colonies and their progenitor cells (CFU-m) were studied in vitro in agar gel. Colony growth required the presence of poke-weed-mitogen-stimulated spleen-conditioned medium. The number of colonies formed was linearly related to both the number of marrow cells plated and the amount of conditioned medium added. In addition, CFU-m were found in both the spleen and peripheral blood. Conditioned medium was also made without plasma, and this resulted in a cloning efficiency greater than that of conditioned medium prepared with plasma. The percentage of CFU-m in DNA synthesis was low (10%), as determined both in vivo and in vitro. Velocity sedimentation revealed that the majority of CFU-m sedimented at 4.3 mm/hr and had a tritiated thymidine (3H-TdR) suicide rate of 1.5 +/- 1.5%. A shoulder on the profile of CFU-m sedimented at approximately 6 mm/hr, with a suicide rate of 79 +/- 2%. Analysis of these data indicated that the majority of CFU-m were not in cycle or were in a long G1 period. The results suggest that CFU-m is a primitive progenitor, possibly closely related to murine splenic colony-forming units (CFU-s), analogous to erythroid bursts and granulocytic colony-forming units.
Coculture studies of 16 untransfused patients with aplastic anemia
Authors: Singer JW , Doney KC , Thomas ED 
Published: October 2019

Abstract
We studied the effects of peripheral blood lymphocytes from 16 untransfused patients with severe aplastic anemia (AA) of diverse etiologies on the growth of granulocyte-macrophage colonies from normal marrows. Normal lymphocytes in our system increased the number of granulocytic colonies by 31 +/- 6% (mean +/- SEM). Lymphocytes from 3 of 16 untransfused AA patients significantly inhibited growth in HLA- matched sibling marrows (-30%, -40%, and -37%; p less than 0.01). Although these results suggest that the majority of cases of AA are not mediated by a coculture-detectable immunologic mechanism, studies using lymphocytes obtained from AA patients before transfusions may detect the subpopulation whose disease is immune-mediated and who may therefore respond to immunosuppressive therapy.
Separation of blast cell and T-lymphocyte progenitors in the blood of patients with acute myeloblastic leukemia
Authors: Minden MD , Buick RN , McCulloch EA 
Published: October 2019

Abstract
The peripheral blood of acute myeloblastic leukemia (AML) patients often contains large numbers of two distinct cell populations, both capable of forming colonies in culture under similar conditions. The first population consists of the precursors of blast cells and has specificity for AML; the second population consists of T-lymphocyte precursors, also found in normal blood. The two progenitor populations can be separated by exploiting the capacity of T-lymphocyte (but not blasts) progenitors to form rosettes with sheep erythrocytes (E rosettes). After E-rosette formation, T-lymphocyte precursors can be removed by centrifugation on Ficoll-Hypaque. Such separation has a number of consequences: (1) Blast progenitors can be detected where unseparated mononuclear preparations have yielded either no colonies or only T-lymphocyte colonies (20 of 21 patients). (2) The stimulator requirements of the blast progenitors change, indicating that cell-cell interactions may take place between blast and T-lymphocyte progenitors. (3) It is feasible to characterize blast and T-lymphocyte precursors independently, even though they may coexist in peripheral blood. This may be important if progenitor properties are attributes contributing to the variance in outcome in AML.
Erythropoietin levels in the course of a patient with erythropoietin- producing renal cell carcinoma and transplantation of this tumor in nude mice
Authors: Toyama K , Fujiyama N , Suzuki H , Chen TP , Tamaoki N , Ueyama Y 
Published: October 2019

Abstract
Erythropoietin was measured by exhypoxic polycythemic mouse method in the course of a 64-yr-old male with renal cell carcinoma associated with erythrocytosis. Serum erythropoietin fluctuated with progression of the disease. Preoperative elevated erythropoietin (0.11 U/ml, p greater than 0.05) subsided after nephrectomy and again increased with developing lung metastasis (0.1 U/ml, p greater than 0.02). Erythropoietin was markedly increased in the tumorous extracts from primary renal cell carcinoma in the kidney (0.2 U/g, p greater than 0.01) and lung metastasis (0.8 U/g, p greater than 0.01). Renal cell carcinoma from the lung metastasis was transplanted into nude mice, resulting in erythrocytosis in some of these mic. In the erythrocytotic mice, erythropoietin was elevated to levels of 0.25--0.9 U/g (p greater than 0.01) in the tumorous extracts and increased (0.67 U/ml, p greater than 0.02) in the serum. These results indicate that this renal cell carcinoma is an erythropoietin-producing tumor, and this tumor has been successfully transplanted in nude mice for the first time.
Principles of blood separation and component extraction in a disposable continuous-flow single-stage channel
Authors: Hester JP , Kellogg RM , Mulzet AP , Kruger VR , McCredie KB , Freireich EJ 
Published: October 2019

Abstract
A single-stage disposable channel and seal that provides for leukocyte and granulocyte collection by continuous-flow cell separation (CFCS) has been designed by the IBM Corporation. This paper describes (1) the separation characteristics of whole blood as it responds to varying gravitational (G) forces and flow rates through the channel; (2) the mechanism by which the buffy coat accumulates and is extracted; (3) the efficiency of extraction; (4) those donor and procedural variables that contribute to the final yield; (5) posttransfusion increment response in patients; and (6) the functional integrity of the cells collected.
Acute lymphoblastic leukemia with pre-B-cell characteristics
Authors: Brouet JC , Preud'homme JL , Penit C , Valensi F , Rouget P , Seligmann M 
Published: October 2019

Abstract
Blast cells from 6 of 50 patients with acute lymphoblastic leukemia (ALL) displayed intracytoplasmic mu chains in the absence of detectable light chains and surface immunoglobulins. These cells also expressed lalike and common ALL antigens. Terminal deoxynucleotidyltransferase was detectable in 2 of 5 cases tested. These blast cells are probably related to early B-cell precursors (pre-B cells). In 4 of 6 cases the disease had a tumoral presentation; the prognostic significance of this new subgroup, which accounts for 20% of patients with non-T non-B ALL, remains to be established.
The protective effect of intraperitoneal splenic autotransplants in mice exposed to an aerosolized suspension of type III Streptococcus pneumoniae
Authors: Dickerman JD , Horner SR , Coil JA , Gump DW 
Published: October 2019

Abstract
Splenosis has been shown to occur after traumatic injury to the spleen. It is postulated that this is the mechanism for the low incidence of bacterial infection in this group of patients when compared to those who undergo splenectomy for other reasons. Therefore, we studied the effect of exposure to an aerosolized suspension of type III Streptococcus pneumoniae on splenectomized mice who had either all or half of their splenic tissue cut up and reimplanted into the abdominal cavity 8 wk prior to bacterial exposure. It was determined that the mortality experience of these two groups of mice was similar to each other and no different from the sham control group, although all three groups had a statistically significant lower mortality experience than the splenectomized control group. This study demonstrates that splenosis in mice can protect against aerosolized bacterial infection.
Mononuclear cell tissue factor: cell of origin and requirements for activation
Authors: Edwards RL , Rickles FR , Bobrove AM 
Published: October 2019

Abstract
Human mononuclear leukocytes generate the procoagulant material tissue factor (TF) following stimulation by endotoxin, mitogens, or antigens in vitro. We have examined tissue-factor generation by mononuclear cell subpopulations prepared in a variety of ways in order to determine the cell of origin of mononuclear cell TF and the conditions necessary for maximal in vitro TF generation. We have also examined the relationship between in vitro TF generation and in vivo or in vitro measures of delayed hypersensitivity in response to identical antigen stimulation. Our results demonstrate that the monocyte is responsible for the bulk of mononuclear cell TF generation in vitro and that adhesion alone is not sufficient stimulation for significant.
Partial purification of thrombopoietin from the plasma of thrombocytopenic rabbits
Authors: Evatt BL , Levin J , Algazy KM 
Published: October 2019

Abstract
Partially purified thrombopoiesis-stimulating activity was prepared from the plasma of thrombocytopenic rabbits using ammonium sulfate precipitation and DEAE cellulose, Sephadex, and carboxymethyl cellulose chromatography. The protein fraction precipitated by an ammonium sulfate saturation of 60%-80%, previously shown to contain thrombopoiesis-stimulating activity, was used as starting material. Column chromatography was carried out at room temperature at pH 5.6. Under these conditions, thrombopoiesis-stimulating activity (thrombopoietin) was retained by DEAE cellulose (0/03 M citrate- phosphate buffer) and carboxymethyl cellulose (0/003 M citrate- phosphate buffer), and eluted with 0.4 M NaCl. Thrombopoietin was retarded by Sephadex G-100; the ratio of the elution volume to the void volume was 1.32:1. Immunoelectrophoretic analysis of partially purified thrombopoietin indicated that following removal of most of the albumin by DEAE chromatography, only proteins with the mobilities of beta- globulins and albumin and traces of other anodally migrating proteins were detectable in the fractions that contained thrombopoiesis- stimulating activity. Thrombopoietin was not dialyzable and was stable from at least pH 5.6 to 7.5. It was approximately 1000-fold purified following sequential chromatography with DEAE and carboxymethyl cellulose. Although the three fractions described reproducibly stimulated thrombopoiesis, as measured by increased levels of selenomethionine-75Se (75SeM) in the circulating platelets, platelet counts did not increase.
Distinct subtype within the spectrum of hairy cell leukemia
Authors: Jansen J , Schuit HR , Schreuder GM , Muller HP , Meijer CJ 
Published: October 2019

Abstract
Most cases of hairy cell leukemia represent malignancies of B cells. However, recent findings suggest that there is a spectrum of functional capacities within the entity hairy cell leukemia. Two patients with hairy cell leukemia, whose malignant cells in the peripheral blood showed both T- and B-cell features, are reported. The malignant cells of the spleens showed only B-cell characteristics. The hairy cells of both patients did not adhere to glass and lacked the la antigen. Both patients showed pronounced polyclonal hypergammaglobulinemia and developed frank leukemic blood pictures after splenectomy. Within the spectrum of hairy cell leukemia, these two cases probably represent a distinct subtype.