bluuebunny/crossref_metadata_2025_split huggingface dataset:
5q- chromosome in acute leukemia with lymphoid morphology and expression of myeloid membrane determinants
Authors: Haas OA , Bettelheim P , Schmidmeier W , Gadner H , Ludwig H , Majdic O , Schulz U 
Published: October 2019

Abstract
We present three patients, two children and one adult, with an unusual type of acute leukemia. Whereas the blast cells showed lymphoid morphology with correlating cytochemical staining, immunological phenotyping exhibited a pure myeloid in one patient and a biphenotypic membrane marker profile in the other two patients. Cytogenetic studies revealed a 5q- chromosome as a common marker and additional individual changes. Two of the patients who were treated according to acute lymphoblastic leukemia (ALL) therapy protocols died without remission five and four weeks after diagnosis, respectively. Despite relapsing several times, another patient survived for over eight years. These three patients seem to represent one new subgroup of leukemias that can only be distinguished from typical ALL by both determination of cell surface markers and cytogenetic analysis.
Abnormal adherence-related functions of neutrophils, monocytes, and Epstein-Barr virus-transformed B cells in a patient with C3bi receptor deficiency
Authors: Buescher ES , Gaither T , Nath J , Gallin JI 
Published: October 2019

Abstract
We evaluated a 3-year-old female patient with leukocytosis, recurrent infections, severe periodontal disease, and a history of delayed separation of the umbilical stump. This patient's polymorphonuclear leukocytes (PMNs) had normal membrane depolarization responses, normal oxygen metabolism, normal granule secretion responses, normal bactericidal activity, and normal C3b rosetting. However, by fluorescent cell analysis and C3bi rosetting, it was determined that her cells lacked the C3bi receptor. In addition, the patient's PMNs showed markedly abnormal chemotaxis, adherence, and aggregation responses, and partial abnormalities were detected in PMN spreading and phagocytosis. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis revealed that the subject's neutrophil cytoplasts were missing a 180,000-dalton moiety. Her monocytes also had defective chemotaxis and failed to adhere and grow normally in culture. Epstein-Barr virus- transformed B cells from the patient lacked an aggregation response to phorbol myristate acetate. Laboratory and clinical evaluations of this patient's mother showed no abnormalities. These studies demonstrate that C3bi receptor deficiency can be associated with functional abnormalities in multiple myeloid cells and that the absence of C3bi receptor is associated with abnormal adherence-related functions of these cells.
The regulation of immunoglobulin synthesis after HLA-identical bone marrow transplantation: VI. Differential rates of maturation of distinct functional groups within lymphoid subpopulations in patients after human marrow grafting
Authors: Lum LG , Seigneuret MC , Orcutt-Thordarson N , Noges JE , Storb R 
Published: October 2019

Abstract
This investigation uses different polyclonal activators of in vitro immunoglobulin production to elicit immunoregulatory profiles of B cells, T cells, T4 cells, and T8 cells from 25 recipients (13 with and 12 without chronic graft-v-host disease [GVHD] ) after HLA-identical marrow transplantation for aplastic anemia or hematologic malignancy. Pokeweed mitogen, Epstein-Barr virus, herpes simplex type 1 virus, and tetanus toxoid were used to induce immunoglobulin production as measured by a plaque assay. Multiple defects in the various lymphoid subsets were found in both groups of patients. There was defective b cell function, lack of T cell or T4 cell subset helper activity, and increased T cell, T4 cell, or T8 cell suppressor activity after stimulation with the various activators. Inconsistent B, T, T4, and T8 cell functions in the marrow graft recipients provide evidence for (a) different functional groups of cells within each subset responsive to different polyclonal activators; (b) a spectrum of immune capabilities within each phenotype lineage; (c) different patterns of immune reconstitution for each lymphocyte subset after marrow grafting; and (d) chronic GVHD altering recovery of in vitro functional responses to the different polyclonal activators.
Alpha zero-thalassemia due to recombination between the alpha 1-globin gene and an AluI repeat
Authors: Nicholls RD , Higgs DR , Clegg JB , Weatherall DJ 
Published: October 2019

Abstract
A form of alpha zero-thalassemia found in subjects of Mediterranean origin has been analyzed by gene mapping and DNA sequencing. Homozygotes have the hemoglobin Bart's hydrops fetalis syndrome, while compound heterozygotes for this defect and alpha+-thalassemia have hemoglobin H disease. It results from a deletion that removes 20.5 kilobases of DNA from within the alpha-globin gene cluster. Sequence data from the regions adjacent to the breakpoint indicate that the recombination event that caused this deletion occurred between the alpha 1-gene and an unusual AluI sequence located between the embryonic zeta genes.
Biologic properties of molecularly cloned and expressed murine interleukin-3
Authors: Hapel AJ , Fung MC , Johnson RM , Young IG , Johnson G , Metcalf D 
Published: October 2019

Abstract
Interleukin-3 (IL-3) (multipotential colony-stimulating factor [multi- CSF]) is an important regulator of hemopoiesis. We recently isolated a cDNA clone of this gene and describe in this manuscript the biologic properties of the expressed gene product. An SV40 expression vector carrying cDNA encoding murine interleukin-3 was constructed so that expression of the IL-3 gene was placed under the control of the SV40 early promoter. When the expression vector was transfected to COS-1 monkey cells, IL-3 activity was secreted into the medium, reaching maximal levels 72 hours after transfection. The IL-3 produced by the COS-1 cells was partially purified using diethylaminoethyl Sephacel and phenyl-Sepharose, and its chromatographic properties were the same as IL-3 produced by the WEHI-3 cell line. The biologic activities of the “expressed” IL-3 include the “induction”of 20-alpha-hydroxysteroid dehydrogenase (20-alpha-SDH) in splenic lymphocytes from nu/nu mice, proliferative activity for 32D cl-23 and FDC-P1 cell lines, and colony- stimulating activity for granulocyte-macrophage, eosinophil, megakaryocyte, natural killer-like, erythroid, and multipotential colony-forming cells from murine fetal liver and adult bone marrow.
Microtubule reassembly in surface-activated platelets
Authors: White JG , Krumwiede M , Sauk JJ 
Published: October 2019

Abstract
It is generally accepted that a circumferential microtubule supports the discoid shape of resting platelets. The fate of the many-coiled polymer following platelet activation, however, has been a subject of considerable debate. Morphological investigations have suggested that the circumferential coils are constricted into tight rings around centrally concentrated organelles during platelet shape change. Biochemical studies employing colchicine-binding assays, on the other hand, have indicated that the bundle of microtubules dissolves almost completely within seconds after activation and reassembles in a new location one to four minutes later. The present study has accepted the latter hypothesis in order to examine the second part of the disassembly-reassembly theory proposed in biochemical studies. Platelets exposed to low temperatures sufficient to remove all microtubules were placed on glass slides and microscope grids to cause surface activation during rewarming. The combined stimuli of rewarming and surface activation might have been expected to cause more rapid assembly than warming alone or activation alone. This was not the case. Reassembly of microtubules during rewarming and simultaneous surface activation was not accelerated. In contrast to the constriction of microtubule rings observed during activation in control platelets, the diameters of coils that developed in chilled platelets one to two hours after rewarming and surface activation were twice those of control cells.
Tac-positive, HTLV-negative, T helper phenotype chronic lymphocytic leukemia cells
Authors: Pandolfi F , De Rossi G , Ranucci A , Bonomo G , Pasqualetti D , Napolitano M , Manzari V 
Published: October 2019

Abstract
In this report we describe an Italian patient with chronic T cell leukemia whose proliferating cells were mature T lymphocytes with a helper phenotype (T helper phenotype chronic lymphocytic leukemia, or Thp-CLL). Unlike other reported cases of Thp-CLL, fresh leukemic cells from this patient were positive with the anti-Tac monoclonal antibody, which recognizes the receptor for interleukin-2 (IL-2). Thus, the phenotype of these cells was similar to that expressed by Japanese patients with adult T cell leukemia (ATL) (OKT3+, OKT4+, OKT8-, Tac+). However, the Italian patient had Thp-CLL, not ATL, since his cells, unlike ATL cells, lacked human T cell leukemia virus (HTLV-I)-related DNA sequences. The Tac receptor, which appears to be modulated in vitro by the anti-Tac antibody, was biologically inactive, since the patient's cells did not respond in vitro to IL-2. In addition, they also failed to demonstrate in vitro functional activities. The clinical course was aggressive, as usual, for both Thp-CLL and ATL. Taking advantage of the description of this case, some similarities and differences between Thp-CLL and ATL are discussed, focusing on the importance of screening of HTLV-I in the differential diagnosis.
Impaired factor X and prothrombin activation associated with decreased phospholipid exposure in platelets from a patient with a bleeding disorder
Authors: Rosing J , Bevers EM , Comfurius P , Hemker HC , van Dieijen G , Weiss HJ , Zwaal RF 
Published: October 2019

Abstract
Platelets from a platelet factor 3-deficient patient, which was first described by Weiss et al (Am J Med 67:206, 1979), were found to be equally impaired in their ability to promote factor X and prothrombin activation. Compared to normal platelets, the patient's platelets showed upon stimulation with thrombin plus collagen a much slower generation and a considerably lower level of platelet prothrombin- and factor X-converting activities. Treatment of stimulated platelets with phospholipases revealed a decreased exposure of negatively charged phospholipid at the outer surface of the patient's platelets, relative to control's. We suggest that the combined impairment of prothrombin- and factor X-converting activities in this patient is due to a defect in the mechanism by which phosphatidylserine becomes exposed at the outer surface of stimulated platelets.
Studies on Platelets
Authors: STEFANINI MARIO , CHATTERJEA JYOTI B. , DAMESHEK WILLIAM , ZANNOS LEDA , SANTIAGO ENRIQUE PEREZ , Salomon Lucy 
Published: October 2019

Abstract
1. Direct transfusions of platelet-rich polycythemic blood, using the multiple syringe technic and siliconized apparatus, were performed in 14 cases of "idiopathic" ("megakaryocytic") and 8 of "secondary" ("amegakaryocytic") thrombocytopenia. Effects on platelet number and function, and on hemorrhagic manifestations were studied.
2. Platelets disappeared very rapidly from the circulation (one-half to twelve hours) in the 12 patients with acute idiopathic thrombocytopenia and in 1 patient with chronic thrombocytopenia with a low platelet count. They disappeared more slowly (twenty-four hours) in the other case of chronic idiopathic thrombocytopenia in which there was a higher platelet count. Essentially similar platelet disappearance curves were obtained in 2 patients, before and after unsuccessful splenectomy. In the patients with secondary thrombocytopenia, the survival of platelets in the circulation was much longer (average forty-eight to ninety-six hours).
3. Behavior of clot retraction and prothrombin activity of serum paralleled closely that of the platelet count. The bleeding time and capillary fragility usually remained improved for twenty-four to forty-eight hours after the platelet count had fallen to the initial level. Likewise, the hemorrhagic manifestations were improved in every case for many hours after the platelet level had returned to its original value. Similar, but less marked beneficial effects on the hemorrhagic manifestations were also obtained with transfusions of fresh blood from donors with normal platelet counts, provided fresh blood was used and precautions taken to prevent excessive loss of platelets.
4. Remissions were observed in 2 patients with idiopathic thrombocytopenia following direct transfusion of polycythemic blood. In both cases, the injected platelets disappeared promptly from the circulation but within twenty-four hours the platelet count began to rise and a long-sustained remission became established. This finding suggests the possibility that polycythemic blood may contain a stimulatory principle which can determine or accelerate a spontaneous remission.
5. Conclusions. The prompt disappearance of injected platelets from the circulation of patients with "idiopathic" thrombocytopenia suggests a humoral destructive mechanism, which is not present in the "secondary" or "amegakaryocytic" cases. This destructive mechanism does not appear to originate in the spleen. Our observations do not exclude an inhibitory effect upon platelet production from the megakaryocytes of the bone marrow. Polycythemic blood may contain a platelet stimulatory factor.
Maintenance of Circulating Red Cell Volume in Rats after Removal of the Posterior and Intermediate Lobes of the Pituitary
Authors: VAN DYKE D. C. , GARCIA J. F. , SIMPSON M. E. , HUFF R. L. , CONTOPOULOS A. N. , EVANS H. M. 
Published: October 2019

Abstract
The anemia which follows hypophysectomy is apparently due to absence of the anterior lobe of the hypophysis as removal of the intermediate and posterior lobes did not change the hemoglobin concentration, hematocrit or volume of circulating red cells.
Correction for Plasma Trapped in the Red Cell Column of the Hematocrit
Authors: CHAPLIN H. , MOLLISON P. L. 
Published: October 2019

Abstract
1. In the hematocrit, the percentage of the red cell column occupied by trapped plasma increases directly with the packed cell volume; this is due to a corresponding reduction of the effective radius of centrifugation.
2. When P.C.V. is determined in Wintrobe hematocrit tubes spun at 3000 r.p.m. in a centrifuge of radius 15 cm., the variation in plasma trapping between one sample and another is much smaller after 55 minutes centrifugation than after 30 minutes. If a 55 minute period is used, variation is so small, even when pathologic bloods are tested, that it is satisfactory to use a calibration curve. Such a curve is presented.
Studies on the Radiosensitivity of Bone Marrow
Authors: PEARCE M. L. , GREENFIELD M. A. , VALENTINE W. N. 
Published: October 2019

Abstract
1. A method for the calculation of "birth rate" of erythrocytes is presented and the dynamics of erythrocyte regeneration are analyzed in terms of a two compartment system.
2. The regeneration of erythrocytes after hemorrhage in irradiated and nonirradiated cats is compared to predicted values.
Erythremic Myelosis (Di Guglielmo's Disease)
Authors: SCHWARTZ STEVEN O. , CRITCHLOW JOAN 
Published: October 2019

Abstract
This article represents a critical attempt to survey the cumulative information of the rare syndrome ordinarily called Di Guglielmo’s disease. The historical background lies predominately in Europe, more particularly in Italy, where the recording of cases has been fairly extensive and where interpretation has been pioneered.
The present study does not presume to be definitive. It attempts to trace the thinking that has gone before, and to define the terms of reference so as not to compound nosologic confusion. It presents examples of cases in the literature acceptable diagnostically by definition. Criteria for the differential diagnosis of acute and chronic erythremic myelosis and erythroleukemia are offered. These three conditions are regarded as variants of reticulo-endotheliosis. This conclusion is based on what is known of the pathogenesis, and on what has been observed clinically and hematologically.
All evaluation is necessarily offered with the reservations that must be held regarding a rare disease of unknown etiology.
Adenine Nucleotide Content of Red Blood Cells of Normal Individuals and of Patients Suffering from Hodgkin’s Disease and Other Diseases
Authors: ROTTINO ANTONIO , HOFFMAN GEORGE T. , ALBAUM HARRY , KRISTAN JOSEPHINE 
Published: October 2019

Abstract
Levels of adenine nucleotides in the blood were determined, using an enzymatic spectrophotometric method.
The levels were determined for a large group of normal persons and a larger group of patients chronically ill from a variety of malignant and degenerative diseases.
The values for adenyl nucleotides were usually normal; a tendency to increased concentration occurred chiefly in anemia.
The varying degrees of increased concentration could not be related to the severity of the anemia or to physical alteration of the red cell. There was probably some relationship to an altered metabolism within the red cell—possibly glycolysis—which in turn might be linked to changes in concentration of electrolytes of the red blood cell, a fact observed in anemia.
Case Report: Possible Paroxysmal Nocturnal Hemoglobinuria with Pronounced Pancytopenia, Reticulocytopenia, and without Hemoglobinuria Simulating Aplastic Anemia
Authors: LETMAN HENNING 
Published: October 2019

Abstract
1. This paper describes a case of chronic severe anemia with very pronounced neutropenia and thrombocytopenia and a varying degree of reticulocytopenia. Crosby’s modification of the Ham test for paroxysmal nocturnal hemoglobinuria proved to be strongly positive. Despite a prolonged period of observation no unquestionable hemoglobinuria was found, but slight hemoglobinemia was found. As the serum bilirubin was normal and there was no reticulocytosis an ordinary hematologic examiation could not have revealed the hemolytic nature of the anemia.
Because of the pronounced pancytopenia and the lack of response to all therapy, the case would presumably have been classified as one of aplastic or "refractory" anemia.
2. The supposition suggests itself that other cases of aplastic or "refractory" anemia and of hemolytic anemia without hemoglobinuria are actually atypical forms of paroxysmal nocturnal hemoglobinuria. The hemoglobinuria in this disease is an immaterial symptom. It should be possible to reveal such atypical cases by means of Ham’s and Crosby’s tests.
3. Pancytopenia is seen not only in the so-called hypersplenic hemolytic anemia, in which the bone marrow is hyperplastic but in congenital hemolytic anemia during crisis and in certain cases of auto-immune acquired hemolytic anemia. It is therefore possible that other forms of hemolytic anemia may be cloaked by a picture of "aplastic anemia."
4. From the standpoint of therapy, it is naturally important to settle the question as to the true nature of the anemia. In congenital and in hypersplenic hemolytic anemia, splenectomy exerts a curative effective; in acquired hemolytic anemia caused by antibodies, treatment with ACTH and cortisone will be effective in many cases; and, finally, in PNH no therapy other than transfusions is presently available.
The Use of Thorium Dioxide Sol (Thorotrast) in the Roentgenologic Demonstration of Accessory Spleens
Authors: LOEB VIRGIL , SEAMAN WILLIAM B. , MOORE CARL V. 
Published: October 2019

Abstract
Experience with the use of thorium dioxide sol (Thorotrast) in the roentgen demonstration of accessory spleens is described. The importance of the application of this technic to the clinical management of patients with hematologic relapse following splenectomy is emphasized and illustrative case reports are presented.
Nine patients were given thorium dioxide in an attempt to demonstrate an accessory spleen. A remission was induced in 1 patient with acquired hemolytic anemia, who had failed to respond to splenectomy, by removal of an accessory spleen demonstrated with thorium dioxide. A second patient with hereditary spherocytosis who relapsed seven years after splenectomy was shown to have an accessory spleen. Seven patients with idiopathic thrombocytopenic purpura who had relapsed following splenectomy were given thorium dioxide and in no case was an accessory spleen found.
Methods: Blood Platelet Counts of the Golden Hamster, Cricetus auratus
Authors: OTTIS KENNETH , TAUBER OSCAR E. 
Published: October 2019

Abstract
Healthy, adult male and female golden hamsters, 3 months of age, showed blood platelet count means of 688,000 ± 141,000 per cu. mm. and 742,000 ± 120,000 per cu. mm., respectively, when direct counts were made with siliconized pipets and with Rees and Ecker fluid as a diluent.
c-fms expression is a molecular marker of human acute myeloid leukemias
Authors: Dubreuil P , Torres H , Courcoul MA , Birg F , Mannoni P 
Published: October 2019

Abstract
The c-fms protooncogene product was identified as the CSF-1 or M-CSF receptor, a polypeptide growth factor that plays a major role in myelomonocytic differentiation. This led us to look for expression of c- fms in fresh acute myeloid leukemia (AML) cells, using Northern blot analysis. c-fms expression was found in the leukemic cells of 28 AML patients, regardless of their stage of differentiation, which was assessed in the French-American-British (FAB) classification. However, the level of c-fms expression was especially high in AML of the M5 stage. High levels of expression were not accompanied by either amplification or rearrangements of the c-fms gene in AML cell DNAs. In contrast, c-fms expression was not found in acute lymphoid leukemias, whether of T or B origin. Thus, c-fms expression appears as a specific molecular marker of leukemogenesis in the myeloid lineage.
Transformation of lymphocytes from immunized Rh(D)-negative subjects by Rh(D) isoantigen
Authors: Varvarigou-Frima A , Mantagos S , Beratis NG 
Published: October 2019

Abstract
Since immune memory in Rh(D)-negative isoimmunized subjects remains through life, even in the absence of measurable anti-Rh(D), we investigated the transformation of lymphocytes from such donors by Rh(D) antigen. The time lapse from the last stimulus was up to 13 years. Mononuclear cells from immunized women were stimulated by Rh(D)- positive erythrocyte stroma. Maximum transformation was observed on the sixth day of culture, with a stroma protein concentration of 8 micrograms/mL of culture medium. The stimulation index (SI) in cells from 11 immunized women was 6.8 +/- 3.1 (mean +/- SD), with a range from 3.1 to 15.0. In five different sets of control cultures, the SI ranged from 0.9 +/- 0.2 to 1.3 +/- 0.4. There was no overlap between stimulated and control cultures. No anti-D could be demonstrated in the serum of four of the 11 immunized cases studied. Also, transformation was observed in mononuclear cells from Rh(D)-negative immunized women with Rh(D)-positive erythrocytes. The findings demonstrate that lymphocytes from isoimmunized Rh(D)-negative subjects maintain the immune memory and are transformed in vitro by the Rh(D) isoantigen.
The Function of the Eosinophile Leukocyte
Authors: VAUGHN JOHN 
Published: October 2019

Abstract
1. Eosinophilia has been produced experimentally in guinea pigs by the injection of extracts of Ascaris suum and histamine, the accompanying histologic changes, particularly the distribution of eosinophils, being noted.
2. The tissues of guinea pigs suffering from a natural eosinophilia have been studied and compared with those of experimental eosinophilia.
3. From these findings a path has been traced which, it is suggested, the eosinophil follows in its course through the body.
4. The function of the eosinophil has been discussed and the hypothesis advanced that its function is the carriage of histamine or a histamine-like toxic material for inactivation.
5. This viewpoint has been considered in relation to the occurrence in man of eosinophilia including eosinophilic infiltration of the lungs.