bluuebunny/crossref_metadata_2025_split huggingface dataset:
Beta O-thalassemia intermedia
Authors: Cividalli G , Kerem H , Ezeckiel E , Rachmilewitz EA 
Published: October 2019

Abstract
Three patients with a relatively mild form of beta O-thalassemia who did not require regular blood transfusions are described. Globin synthesis was studied by gel filtration and urea-carboxymethylcellulose chromatography of stroma-free hemolysates prepared from peripheral blood and bone marrow cells labeled in vitro with 14C-leucine. gamma/alpha Synthetic ratios in peripheral blood were in the same range as in patients with the severe clinical form of beta O-thalassemia, while gamma/alpha synthetic ratios in bone marrow cells were higher than in that group of patients. The size of the free alpha-chain pool measured in one case was smaller than in other patients with “classical” Cooley anemia. It is concluded that the severity of the clinical course in beta O-thalassemia does not correlate with the imbalance in alpha verus gamma chain synthesis in peripheral blood and is determined by the synthetic ratio in bone marrow cells, where the bulk of hemoglobin synthesis takes place.
Modified method of erythropoietin (ESF) bioassay in vitro using mouse fetal liver cells. I. Effect of serum iron on 59Fe incorporation into heme
Authors: de Klerk G , Kruiswijk C , Hart AA , Goudsmit R 
Published: October 2019

Abstract
Investigations on the mouse fetal liver cell bioassay for erythropoietin (ESF) have revealed that iron present in test sera significantly dilutes the radiolabel (59Fe) and thus decreases 59Fe incorporation into heme. A method of correction for the influence of iron on the dose-response relationship of human sera is presented. Application of this method made it possible to assay human sera up to culture concentrations of 150 microliter/ml. It was shown that a corrected serum dose-response curve showed parallelism to the curve of an ESF standard preparation. This suggests similarity of the active principles and allows valid estimation of a potency ratio.
Modified method of erythropoietin (ESF) bioassay in vitro using mouse fetal liver cells. II. measurement of ESF in human serum
Authors: de Klerk G , Hart AA , Kruiswijk C , Goudsmit R 
Published: October 2019

Abstract
A modification of the mouse fetal liver cell bioassay for erythropoietin (ESF) that allows quantitative detection of ESF in human serum is described. The modification consists of (1) correction for the effect of serum iron on 59Fe incorporation into heme and (2) the use of an “internal standard,” i.e., a standard ESF preparation dissolved in the assayed test serum. As a result of this modification the statistical method of bioassay analysis had to be changed fundamentally. The mean serum concentration of ESF measured in 20 normal males was 48 microunit/ml, as compared to 29 microunit/ml in 18 females. The difference was significant at p = 0.017. The stimulatory activity of a human serum on 59Fe incorporation into heme could be neutralized by an anti-human ESF immune serum.
Proliferative state of blast cell progenitors in acute myeloblastic leukemia (AML)
Authors: Minden MD , Till JE , McCulloch EA 
Published: October 2019

Abstract
Peripheral blood from patients with acute myeloblastic leukemia (AML) contains cells capable of giving rise to colonies in culture when stimulated by media conditioned by leukocytes (LCM) in the presence of phytohemagglutinin (PHA). Two types of colonies are recognized with high frequency: The first grows in the presence of low concentrations of PHA LCM, have a blast-like morphology, and are numerically correlated with morphologically identified blast cells. The second requires either high PHA LCM concentrations or PHA alone with or without 2-mercaptoethanol and consists of cells capable of forming rossettes with sheep erythrocytes and resembles. T-lymphocyte colonies from normal blood. Precursors of blast cell colonies from 15 leukemic patients were tested for cycle state, using either the 3H-thymidine or hydroxyurea techniques. All were found to have a high proportion of cells in the S phase of the cycle. In contrast, T lymphocyte precursors from three normal individual were quiescent. The data are consistent with the maintenance of the leukemic blast cell populations by the proliferative activity of a small subpopulation of blasts.
Fibrin formation: effect of calcium ions
Authors: Brass EP , Forman WB , Edwards RV , Lindan O 
Published: October 2019

Abstract
Using laser fluctuation spectroscopy, a technique that measures particle size change in solution, the kinetics of fibrin clot formation from fibrinogen can be studied. With this technique the effect of calcium on the three distinguishable phases of clot formation, (1) proteolysis of fibrinogen, (2) fibrinogen-fibrin monomer complex formation, and (3) fibrin monomer polymerization, were investigated. Only a small change in the length of the induction period that results from the fibrinogen-fibrin monomer interactions was observed. However, there was a marked increase in the rate of fibrin monomer polymerization in the presence of calcium ions. These data show that calcium decreases the time required for fibrin formation from fibrinogen by markedly accelerating the phase of fibrin monomer polymerization.
Colony formation in vitro by leukemic cells in acute lymphoblastic leukemia (ALL)
Authors: Smith SD , Uyeki EM , Lowman JT 
Published: October 2019

Abstract
An assay system in vitro for the growth of malignant lymphoblastic colony-forming cells (CFC) was established. Growth of malignant myeloblastic CFC has been previously reported, but this is the first report of growth of malignant lymphoblastic CFC. Established assay systems in vitro have been very helpful in elucidating the control of growth and differentiation of both normal and malignant bone marrow cells. Lymphoblastic CFC were grown from the bone marrow aspirates of 20 children with acute lymphoblastic leukemia. Growth of these colonies was established on an agar assay system and maintained in the relative hypoxia (7% oxygen) of a Stulberg chamber. The criteria for malignancy of these colonies was based upon cellular cytochemical staining characteristics, the presence of specific cell surface markers, and the ability of these lymphoid cells to grow without the addition of a lymphoid mitogen. With this technique, specific nutritional requirements and drug sensitivities can be established in vitro, and these data may permit tailoring of individual antileukemic therapy.
Absence of beta mRNA in beta0-thalassemia in Kurdish Jews
Authors: Ramirez F , Starkman D , Bank A , Kerem H , Cividalli G , Rachmilewitz EA 
Published: October 2019

Abstract
We report the characterization of the amount of beta mRNA in a Kurdish Jewish population with beta0-thalassemia using the same methods employed for characterization of the Catania and Ferrara beta0 patients. We found very low amounts of beta mRNA sequences, consistent with the presence of beta0-thalassemia of the beta mRNA-negative population type. In addition, no globin gene deletion was detected that could account for the absence of beta mRNA.
Plasma cell leukemia (PCL): A report on 15 patients
Authors: Woodruff RK , Malpas JS , Paxton AM , Lister TA 
Published: October 2019

Abstract
Fifteen patients presenting with plasma cell leukemia (PCL) are reported in detail. The clinicopathologic features of PCL differ from typical myeloma and resemble those of acute leukemia: patients with PCL have less bone disease but a much higher incidence of organomegaly and tissue infiltration as well as diffuse marrow involvement and more pronounced pancytopenia. One of the reported patients developed meningeal plasma cell leukemia and is reported in detail. Cytomorphologic assessment of PCL cells showed nuclear immaturity and obvious nuclear/cytoplasmic asynchrony. Despite the use of cytotoxic agents known to be effective in myeloma, the prognosis in PCL is poor, and the median survival of the reported patients was only 2 mo.
Atypical lymphoid leukemia in ataxia telangiectasia
Authors: Levitt R , Pierre RV , White WL , Siekert RG 
Published: October 2019

Abstract
We observed two sisters with ataxia telangiectasia, one of whom developed an atypical subacute lymphocytic leukemia characterized by atypical lymphocytes and absence of palpable lymphadenopathy or hepatosplenomegaly. The lack of organomegaly in this patient may have been due to the underlying ataxia telangiectasia, which was associated with lymphoid hypoplasia. Cytogenetic studies showed a marker chromosome 14 [t(14q11:14q34)] in both patients. The sister with leukemia had other complex chromosomal aberrations in addition to the marker chromosome 14 that were stable for more than 14 mo before the patient's death from complicating infection. The development of atypical T cell leukemia has not been previously described in ataxia telangiectasia. This case further illustrates the interesting interrelationships amoung immunosuppressed states, development of lymphoid malignancy, and an emerging pattern of a propensity to chromosome 14 abnormalities in various lymphoid malignancies.
Deletion of the long arm of chromosome 20 [del(20)(q11)] in myeloid disorders
Authors: Testa JR , Kinnealey A , Rowley JD , Golde DW , Potter D 
Published: October 2019

Abstract
Detailed clinical and cytogenetic studies were performed in five patients who had abnormal hematopoiesis and an acquired deletion of an F-group chromosome. Cytogenetic analyses, with banding techniques, of cells from bone marrow, spleen, or unstimulated peripheral blood showed a partial deletion of the long arm of one chromosome 20 [del(20)(q11)] in all five patients. Three patients had myeloproliferative disorders of uncertain classification, the fourth had possible preleukemia, and the fifth had acute myelomonocytic leukemia. Although the five cases showed certain similarities, the clinical and hematologic findings seen with the 20q- abnormality were not specific. None of the patients showed evidence of polycythemia vera or idiopathic acquired refractory sideroblastic anemia, two diseases previously associated with the 20q-. Our studies indicate that the 20q- abnormality is not limited to diseases primarily affecting erythropoiesis but that it can be found in the broader spectrum of myeloid disorders. In polycythemia vera, the 20q- has sometimes been regarded as a possible result of previous therapy with cytotoxic agents; however, four of our patients were untreated when the deletion was first noted.
Leukemic lymphosarcoma (LLS) with monoclonal IgM: idiotypic specificity on the cell surface and in the cytoplasm of lymphosarcoma cells
Authors: Sugai S , Hirose Y , Konaka Y , Konda S 
Published: October 2019

Abstract
A case of leukemic lymphosarcoma with a small amount of monoclonal IgM in the serum is described. Immunologic investigations showed that about 10% of the abnormal cells formed EAC rosettes, that most of these cells had IgMlambda on the cell surface, and that 40% of them contained IgMlambda in the cytoplasm. Monoclonal IgM was detected in the 19S fraction of the serum despite the lack of monoclonal change as determined by serum electrophoresis or immunoelectrophoresis. The common idiotypic specificity was found in the monoclonal serum IgM, surface Ig, and cytoplasmic Ig of lymphosarcoma cells using antiidiotypic antiserum prepared against purified serum IgM. After chemotherapy, cells with idiotypic determinants disappeared and the serum IgM changed from a monoclonal to a polyclonal distribution.
Red cell diphosphoglycerate mutase. Immunochemical studies in vertebrate red cells, including a human variant lacking 2,3-DPG
Authors: Peterson LL 
Published: October 2019

Abstract
Diphosphoglycerate mutase (DPGM) was purified to homogeneity from human erythrocytes. The enzyme and Freund adjuvant were injected into chickens and yielded a monospecific precipitating antibody. Radial immunodiffusion with this antibody was used to measure the amount of DPGM in hemolysates from human adult and cord red cells. Dog, rabbit, rat, chicken, and goat red cells all had DPGM during the neonatal period, but goat adult red cells had no detectable enzyme. Single bands with no spurs were present on Ouchterlony plates in which human hemolysate was placed adjacent to hemolysates from the other species tested. The amount of human red cell DPGM did not differ between young and old cells separated by centrifugation. Red cells from a patient with a DPGM genetic variant who had erythrocytosis and no detectable enzyme activity contained a reduced amount of DPGM as determined by radial immunodiffusion. The abnormal DPGM differed from normal by immunoelectrophoresis and in stability as measured by the amount of crossreacting material in young versus old erythrocytes.
Augmentation of fetal hemoglobin (HbF) synthesis in culture by human erythropoietic precursors in the marrow and peripheral blood: studies in sickle cell anemia and nonhemoglobinopathic adults
Authors: Kidoguchi K , Ogawa M , Karam JD , Martin AG 
Published: October 2019

Abstract
We cultured marrow and peripheral blood erythropoietic precrusors in methylcellulose clonal assay and measured the synthetic rates of HbA, A2, F, and S in patients with and without sickle cell anemia. Hb was labeled with 14C-amino acid in culture, separated by slab gel isoelectric focusing techniques, and quantitated by autoradiographic methods. Comparison of marrow late (CFU-E) and early (BFU-E) precursors from patients without hemoglobinopathies showed that preferential synthesis of HbF is limited to early precursors. Simultaneous examinations of Hb synthesis by blood and marrow early erythropoietic precursors confirmed the similarity of the biosynthetic capabilities of the precursors from the two sources. Increasing concentrations of erythropoietin (Ep) in culture corresponded with increases in the percentages of HbF synthesized by blood BFU-E of normal individuals. HbF biosynthesis by blood BFU-E from sickle cell anemia patients was significantly higher than that synthesized by nonanemic individuals and showed significant individual variations. HbF synthesis in patients with sickle cell anemia was partially dependent on Ep concentrations in culture. Cell culture of circulating erythropoietic precursors in man appears to provide a unique tool for studying the control mechanisms of Hb synthesis in man.
Successful use of multiagent immunosuppression in the bone marrow transplantation of sensitized patients
Authors: Parkman R , Rappeport J , Camitta B , Levey RH , Nathan DG 
Published: October 2019

Abstract
Of 23 patients with severe aplastic anemia, 17 were sensitized to histocompatibility antigens of HLA-A, -B, and -D loci-identical potential sibling donors as determined by cell-mediated lysis (CML) assays in vitro. Antibody-dependent sensitization was detected in 3 patients, antibody-independent cellular sensitization in 11, and both in 3. Fourteen sensitized patients were transplanted after initial multiagent immunosuppression consisting of rabbit anti-human thymocyte serum, procarbazine, and cyclophosphamide, eleven with a CML-positive donor and three with a CML-negative donor. Engraftment was achieved in each of 13 patients who were evaluable, and only 2 ultimately rejected their marrow grafts, 1 with subsequent return of his own marrow function. Five patients without evidence in vitro of sensitization were transplanted after immunosuppression with cyclophosphamide alone; none of these rejected their grafts. These studies show that sensitized bone marrow transplant recipients can be successfully transplanted after optimal donor selection and multiagent immunosuppression.
Nuclear RNA sequences coding for alpha and beta globins in erythroid cells: evidence for multiple intermediate molecules
Authors: Farace MG , Ullu E , Fantoni A , Rossi GB , Cioe L , Dolei A 
Published: October 2019

Abstract
The poly (A)-containing nuclear RNA from dimethylsulfoxide-induced Friend leukemia cells was fractionated by acrylamide gel electrophoresis in denaturing conditions and analyzed for alpha and beta globin RNA sequences. The results indicate that nuclear RNA contains one species of large-size RNA (0.6 X 10(6) daltons), which is the putative precursor for beta globin mRNA only. In addition, it was shown by electrophoretic analysis that the complex of RNA molecules not resolved by sucrose gradient centrifugation (11S) comprises sequences of decreasing size (0.34, 0.28, and 0.26 X 10(6) daltons), which might be the precursors of alpha and beta globin mRNA.
Results of treatment of Ph'+ chronic myelogenous leukemia with an intensive treatment regimen (L-5 protocol)
Authors: Cunningham I , Gee T , Dowling M , Chaganti R , Bailey R , Hopfan S , Bowden L , Turnbull A , Knapper W , Clarkson B 
Published: October 2019

Abstract
Thirty-seven patients with Philadelphia-chromosone-positive (Ph′+) chronic myelogenous leukemia who were untreated or minimally pretreated were entered on the L-5 protocol. This protocol consisted of sequential treatment with splenic irradiation, splenectomy, arabinosylcytosine and 6-thioguanine, and L-asparaginase. Maintenance therapy was hydroxyurea or a multiple-drug regimen. The median survival of the 37 patients is 50 mo. Twelve patients showed a temporary reduction in the percentage of Ph′+ marrow metaphases to less than one-third of the initial values and in 7 of these patients none were found. The duration of the Ph′+ chromosome reduction ranged from 1 to 43 mo. The median survival of the responders has not yet been reached. It is concluded that whereas overall survival is not appreciably extended, patients who have a reduction in Ph′+ cells in the marrow may survive longer than the average; also, the reduction occurs most frequently in patients who have relatively small spleens at diagnosis. The reduction is difficult to maintain, and it may be reinduced in some patients with intensive chemotherapy.
Hb F production of endogenous colonies of polycythemia vera
Authors: Papayannopoulou T , Buckley J , Nakamoto B , Kurachi S , Nute PE , Stamatoyannopoulos G 
Published: October 2019

Abstract
Fetal hemoglobin was studied in endogenous colonies produced in plasma clot and methylcellulose cultures of circulating progenitors from patients with polycythemia vera (PV). Analysis of globin chain synthesis showed that gamma chains constituted from 13% to 42% of the non-alpha chains produced in cultured cells, whereas from 27% to over 50% of the endogenous colonies contained Hb F, as indicated by the fluorescent antibody probe. Since the endogenous colonies in PV cultures originate from the abnormal PV clone, the findings provide direct evidence that a single pluripotent stem cell can have committed progeny that differ in their expressions of the Hb F production program.
Oxidative metabolic responses of rabbit pulmonary alveolar macrophages
Authors: Boxer LA , Ismail G , Allen JM , Baehner RL 
Published: October 2019

Abstract
During phagocytosis of opsonized lipopolysaccharide-coated paraffin oil droplets, rabbit alveolar macrophages reduced nitroblue tetrazolium, which effect was in part inhibitable with the use of superoxide dismutase. Exposure of cytochalasin-B-treated rabbit alveolar macrophages to opsonized zymosan led to the generation of superoxide, as quantitated by ferricytochrome C reduction. It was found that nitroblue tetrazolium in the presence of ferricytochrome C could in turn serve as scavenger of superoxide during stimulation of cytochalasin-B-treated rabbit alveolar macrophages. Following challenge with either opsonized zymosan or the membrane perturbant digitonin, rabbit alveolar macrophages released hydrogen peroxide into the extracellular medium. Employment of the surface membrane stimulant phorbol myristrate acetate led to activation of the hexose monophosphate shunt, which activity could be further enhanced in the presence of superoxide dismutase or attenuated in the presence of catalase. These studies demonstrate that rabbit alveolar macrophages release superoxide and hydrogen peroxide during surface membrane perturbation. In turn, hydrogen peroxide generation can stimulate the hexose monophosphate shunt.
Splenectomy in myeloid metaplasia
Authors: Silverstein MN , ReMine WH 
Published: October 2019

Abstract
Between 1960 and 1977, 50 patients with agnogenic myeloid metaplasia were splenectomized. Twenty-five of 26 patients with painful splenomegaly, 4 of 9 patients with refractory hemolytic anemia, 4 of 10 patients with refractory thrombocytopenia, and 4 of 4 patients with portal hypertension showed significant benefit from the procedure. There were five immediate postoperative deaths. Four of these deaths occurred early in our series of splectomies for myeloid metaplasia before 1970. Only one death has occurred in the last 21 patients operated on. Survival following splenectomy averaged 25.5 mo.
Evidence for production of oxidizing radicals by the particulate O-2- forming system from human neutrophils
Authors: Tauber AI , Gabig TG , Babior BM 
Published: October 2019

Abstract
The particulate O-2-forming system from human neutrophils was found to oxidize methional and 2-keto-4-methylthiobutyric acid (KMB) to ethylene, indicating the formation by this system of strongly oxidizing radicals. Conforming this interpretation was the observation that ethylene production was inhibited by the radical scavengers benzoate, ethanol, and mannitol. Ethylene production was also sharply reduced by superoxide dismutase, implicatin O-2 as a precursor of oxidizing radicals. In our system catalase only partially inhibited ethylene generation from either methional or KMB, suggesting that oxidizing radicals are generated at least in part by the reacton of O-2 with compounds other than H2O2. We propose that in neutrophils oxidizing radicals are formed in a reaction between O-2 and a peroxide according to the following equation: O-2 + ROOH leads to RO . + OH- + O2, in which ROOH may be hydrogen peroxide, an alkyl peroxide, or an acyl peroxide (i.e., a peroxy acid).
Improved prognosis for patients with mediastinal lymphoblastic lymphoma
Authors: Weinstein HJ , Vance ZB , Jaffe N , Buell D , Cassady JR , Nathan DG 
Published: October 2019

Abstract
Patients with diffuse lymphoblastic lymphoma (which includes convoluted lymphocytic lymphoma) with mediastinal involvement have predictable progression of disease to a leukemic phase that is cytologically indistinguishable from acute lymphoblastic leukemia (ALL). Therefore we treated 12 patients with diffuse lymphoblastic lymphoma involving the mediastinum with therapy that is effective in ALL. Treatment consisted of intermittent combination chemotherapy with adriamycin and preventive central nervous system therapy (craniocervical irradiation and intrathecal methotrexate). Mediastinal irradiation was given either for initial respiratory distress or to patients who had incomplete regression of disease following induction chemotherapy. Eleven patients achieve complete remission. With a median follow-up of 41 mo, and using life table analysis, 86% of these patients have remained in continuous complete remission. The results of this study demonstrate the efficacy of treating diffuse lymphoblastic lymphoma with mediastinal presentation as a disseminated lymphoid malignancy.
Emergence of a cell line with extreme hypodiploidy in blast crisis of chronic myelocytic leukemia
Authors: Como RM , Graze PR 
Published: October 2019

Abstract
Cytogenetic studies in a patient with chronic myelocytic leukemia (CML) demonstrated the emergence of an extremely hypodiploid cell line at the time of blast crisis, a modal chromosome number of 35, with the modal karyotype 35,XY, -3, -4, -5, -7, -9, -11, -12, -13, -15, -16, -17, -19, -20, -22, + t(9;22) (q34;q11, + Mar1, + Mar2, + Mar3. Giemsa-banding confirmed complex chromosome rearrangements and demonstrated distinct banding patterns for the marker chromosomes. Cytologic characteristics of the leukemia blasts were predominantly myeloid. There was no important clinical response to chemotherapy, including vincristine and prednisone, or to radiotherapy.