bluuebunny/crossref_metadata_2025_split huggingface dataset:
Platelet Functional Abnormalities: A Report of Two Familial Defects in Interaction Between Collagen and Platelets and ADP Release
Authors: PAPAYANNIS A. G. , WATSON-WILLIAMS E. J. , ISRAËLS M. C. G. 
Published: October 2019

Abstract
Two families have been studied, members of which have a lifelong hemorrhagic diathesis. In both families, a platelet functional abnormality has been found, with the coagulation mechanism intact. Findings common to both families were a prolonged bleeding time, reduced platelet adhesiveness, absence of the second wave of aggregation with ADP and reduced ADP release from platelets. The clot retraction, platelet factor 3 availability and aggregation of platelets with ADP in a final concentration of 1.0 and 20.0 µM were normal. The differences between the families were the platelet morphology, the in vitro adherence of platelets to collagen fibrils, the collagen-induced aggregation, and the thrombin-induced aggregation. In one of them, the Windle family, the platelets were abnormally large in size and showed a delayed adherence to collagen fibrils. The aggregation with collagen was either absent or feeble, and with thrombin, showed an abnormal pattern. In the other, the Hughes family, the platelets were morphologically normal and adhered to collagen fibrils normally. The aggregation with collagen initiated normally but usually did not proceed to completion and the aggregation with thrombin was normal. It appears that in the Windle family the main abnormality concerns the reaction between platelet and collagen and the condition is associated with impaired ADP-release mechanism and abnormal thrombin-platelet reaction, and in the Hughes family the ADP-release mechanism of platelets is defective. On the basis of current information, including that from these families, a classification of the various platelet functional abnormalities and methods for characterizing them is proposed.
Congenital Dyserythropoietic Anemia Type II: Ultrastructural and Radioautographic Studies of Blood and Bone Marrow
Authors: Wong Kwan Yuen , Hug George , Lampkin Beatrice C. 
Published: October 2019

Abstract
A 12-yr-old white girl with congenital dyserythropoietic anemia (CDA) type II was studied. Excessive cytoplasmic membranes (appearing like "double membranes") were found in the majority of the normoblasts. There was marked decrease in the uptake of tritiated thymidine in the binucleated normoblasts as demonstrated by radioautography. The results suggest that the cells with more severe structural cytoplasmic abnormalities and/or decreased DNA synthesis are destroyed within the bone marrow, and the circulating red cells are derived from a less abnormal population of precursors.
Fine Structure of the Red Pulp of the Spleen in Hereditary Spherocytosis
Authors: MOLNAR ZELMA , RAPPAPORT HENRY 
Published: October 2019

Abstract
The spleens from two children and one adult with hereditary spherocytosis were studied in the electron microscope. Stagnation of the erythrocytes within the splenic cords is attributable to their lack of plasticity as evidenced by the absence of bilobed, tailed, or squeezed forms in transit through the walls of the sinuses. In contrast to the sections studied by conventional light microscopy, the splenic sinuses in hereditary spherocytosis were not "empty," but contained red blood cells, the majority of which had lost their hemoglobin content. Cordal macrophages were increased in all three cases and were abundant in the splenic cords of the adult patient, causing a further impediment to the rapid passage of erythrocytes. Macrophages, and, to a lesser degree, sinus endothelial cells contained the products of hemoglobin breakdown. The macrophages showed active erythrophagocytosis. Sinus endothelial cells rarely contained intact red blood cells, but showed pronounced pinocytotic activity, a probable mechanism of hemoglobin incorporation. Platelets within the endothelial cells of the sinuses were much more frequently seen in the three cases of hereditary spherocytosis than in control spleens. The presence of ferritin in platelets suggests that they too may play a role in clearing the end products of hemolysis from the spleen.
IgA Pyroglobulin, Hyperviscosity Syndrome and Coagulation Abnormality in a Patient With Multiple Myeloma
Authors: Sugai Susumu 
Published: October 2019

Abstract
A 48-yr-old woman with a bleeding diathesis and complaining of blurred vision, headache, paresthesias, and vomoting was diagnosed as having IgA, kappa type multiple myeloma. Markedly increaesed serum viscosity was noted. After plasmapheresis the signs and symptoms of high serum viscosity disappeared. Pyroglobulin was found in the M protein. The M protein had a prominent 15.5S peak and smaller 17.0 and 20S peaks, which decreased to a 6.7S peak after in vitro reduction with 0.1 M 2-mearcptoethanol, 2-mercaptoethylamine hydrochloride, or glutathione. The serum viscosity was decreased by treatment with these reducing agents. Pyroglobulin formation was presumably a noncovalent intermolecular reaction, as it was inhibited by 5.0 M guanidine hydrochloride, acidification and alkalinization, but not by reduction with sulfhydryl reagents. The M protein also had an antithrombin activity causing interference with the conversion of fibrinogen to fibrin and the phenomenon of gelification noted on blood clotting.
Hemopoiesis in Pituitary Dwarfs Treated With Human Growth Hormone and Testosterone
Authors: Jepson J. H. , McGarry E. E. 
Published: October 2019

Abstract
Prepubertal hypopituitary dwarfs are anemic and have reduced levels of erythropoietin-stimulating factor (ESF) in the urine. Treatment with human growth hormone (HGH) in doses sufficient to produce linear growth and weight gain induces bone marrow lymphocytosis, increased erythropoiesis indicated by an increase of the red cell mass (RCM) and improved iron kinetic studies. This was associated with increased concentrations of ESF in the urine, increased transferrin levels, and expansion of the plasma volume. The combination of testosterone and HGH increased further erythropoiesis but could not be specifically ascribed to testosterone, since the continued action of HGH could be responsible for the observed effects. The excretion of ESF was not increased further. Testosterone did, however, maintain ESF excretion on deletion of HGH from the regimen, suggesting at least one common mode of action for these hormones in the hypoanabolic subject. In contrast, testosterone failed to maintain the plasma volume increase induced by HGH and did not further effect the observed bone marrow lymphocytosis.
Serum Requirement for Apoferritin Induction in Rat Liver Slices
Authors: Smith Lester , Fineberg R. A. 
Published: October 2019

Abstract
In studies of ferritin induction, liver slices failed to respond favorably to iron added directly to the suspending medium unless serum was added. The addition of 5 µg Fe/ml to slices in whole serum accelerated the incorporation of radioleucine into ferritin 2.5-fold. The favorable effect of serum on iron-induced ferritin synthesis was correlated with its attenuation of the toxic effects of the iron on protein synthesis; the serum was also found to diminish the uptake of iron by the liver cells. Transferrin, purified from the serum by ion exchange, failed to mediate the iron induction of apoferritin while albumin, completely freed from transferrin as monitored with radioiron, was effective. Albumin, apparently by limiting the uptake of excess iron by the liver cells, modulates the nonspecific toxic effects of iron on protein synthesis and thereby permits the specific induction by iron of the biosynthesis of apoferritin.
Identification of the Bone Marrow Colony Mononuclear Phagocyte as a Macrophage
Authors: Cline Martin J. , Warner Noel L. , Metcalf Donald 
Published: October 2019

Abstract
Bone marrow colony-derived mononuclear phagocytes are macrophages on the basis of morphology, glass adherence, the phagocytosis of a range of particles including erythrocytes, yeast, and bacteria, and the possession of receptors for IgG of predominantly IgG2a specificity.
IgE Multiple Myeloma: A Report of the Third Case
Authors: Fishkin Ben G. , Orloff Natalie , Scaduto Louis E. , Borucki David T. , Spiegelberg Hans L. 
Published: October 2019

Abstract
The third known case of IgE multiple myeloma is described. The patient was a 65-yr-old woman who died 11 mo after a diagnosis of multiple myeloma was made by a surgical biopsy of the left iliac crest. Her serum contained approximately 2.7 g/100 ml of a paraprotein of mid-γ mobility. The isolated anomalous protein gave a reaction of identity with the IgE myeloma protein of the second reported case when reacted with a specific IgE antiserum. The light-chain type of the myeloma protein was κ in contrast to λ of the other two IgE myeloma proteins. Her clinical manifestations differed from the two previously reported cases in several respects. She had neither plasma cell leukemia nor overt Bence Jones proteinuria and developed rapidly progressive widespread osteosclerotic lesions that were associated with bone pain.
Possible Thrombosthenin Defect in Glanzmann’s Thrombasthenia
Authors: Booyse Francois , Kisieleski Donna , Seeler Ruth , Rafelson Max 
Published: October 2019

Abstract
Microquantitation of thrombosthenin by single radial immunodiffusion in conjunction with a specific immunohistochemical antibody staining technique has indicated an immunologic defect or absence of the surface-localized thrombosthenin (S-thrombosthenin) in platelets from Glanzmann’s thrombasthenic patients. In addition, ADP- and ATP-induced changes of the surface of normal platelets could not be demonstrated with Glanzmann’s thrombasthenic platelets.
Immunologic Studies of Antihemophilic Factor (AHF, Factor VIII). III. Comparative Binding Properties of Human and Rabbit Anti-AHF
Authors: Hoyer Leon W. 
Published: October 2019

Abstract
The binding properties of human and rabbit antibodies to antihemophilic factor (AHF, factor VIII) have been compared in experiments that sought the basis for their different specificities and secondary properties. Agarose gel filtration demonstrated the formation of stable complexes of AHF with rabbit anti-AHF but not with human anti-AHF. Measurement of bound radiolabeled rabbit anti-AHF provides a very sensitive assay for the AHF antigen that is present in normal and hemophilic plasmas but that is markedly reduced in plasmas from patients with von Willebrand’s disease.
Porphyrin Synthesis by Cultured Lymphocytes
Authors: Josephson Alan S. , Levere Richard D. , Lowenthal Ivan , Swerdlow Fred , Ginsberg Mark 
Published: October 2019

Abstract
Peripheral lymphocytes were cultured in the presence and absence of phytohemagglutinin (PHA). Porphyrin synthesis was visually estimated by fluorescence and quantitated by fluorometry. No visual fluorescence was noted in cells cultured with or without PHA. Addition of δ-aminolevulinic acid (ALA) to cultures of either transformed or nontransformed lymphocytes resulted in fluorescence, indicating porphyrin synthesis. Certain sex steroid metabolites stimulate porphyrin synthesis by induction of the rate-limiting enzyme ALA synthetase. Erythropoietin also increases the rate of heme synthesis. Phytohemagglutinin-transformed and nontransformed cells were cultured with the addition of either 11-keto pregnanolone or erythropoietin, and neither agent induced porphyrin synthesis. Only when ALA was added to the culture medium was the measured fluorescence statistically greater than in control cultures. These studies indicate that the enzymes of porphyrin synthesis distal to ALA synthetase, the rate-limiting enzyme, are present in all cultured lymphocytes. The rate-limiting enzyme is not induced by PHA transformation, even in the presence of a 5-β-H steroid metabolite or erythropoietin.
Ultrastructural Studies in Acute Promyelocytic Leukemia
Authors: Tan Henry K. , Wages Bettye , Gralnick Harvey R. 
Published: October 2019

Abstract
In this investigation, the cells of eight patients with acute promyelocytic leukemia were followed by light and electron microscopy. Promyelocytes from untreated patients were filled with large, splinter-shaped granules. The granules were lysosomes, with some showing the ultrastructural features of Auer bodies. In cases responsive to chemotherapy, promyelocytes contained only infrequently splinter-shaped lysosomes, while most lysosomes were of more uniform size and shape. The persistence of large numbers of splinter-shaped lysosomes within promyelocytes was associated with episodes of disseminated intravascular coagulation less responsive to heparin treatment. Promyelocytes from patients in remission were similar to promyeloctyes from nonleukemic bone marrow. The ultrastructural differences in lysosome morphology provide a better criterion for distinguishing malignant from normal promyelocytes than previously detectable by light microscopy alone.
Studies on Human Erythrocyte Nucleotide Metabolism. II. Nonspherocytic Hemolytic Anemia, High Red Cell ATP, and Ribosephosphate Pyrophosphokinase (RPK, E.C. 2.7.6.1) Deficiency
Authors: Valentine William N. , Anderson Helen M. , Paglia Donald E. , Jaffé Ernst R. , Konrad Patricia N. , Harris Susan R. , Kürschner Klaus K. 
Published: October 2019

Abstract
A 29-yr-old black woman was found to have a long-standing, nonspherocytic hemolytic disorder associated with a marked reduction in the activity of erythrocyte ribosephosphate pyrophosphokinase (RPK, PRPP synthetase, E.C. 2.7.6.1). Although the patient’s erythrocytes had about 50% of the average RPK activity of normal mature human erythrocytes, this level represented only about 20-30% of the activity in comparable reticulocyte-rich blood samples from patients with other types of hemolytic anemias. The concentrations of adenosine triphosphate adenosine diphosphate, adenosine monophosphate and, therefore, of total adenine nucleotides in her erythrocytes were markedly increased, even well above the levels in extracts of comparable reticulocyte-rich blood samples. ATPase activity was increased three- to fourfold, consistent with the reticulocytosis. Adenylate kinase and adenine phosphoribosyltransferase activities were normal. The activities of all enzymes of the Embden-Meyerhof and hexose monophosphate shunt pathways and enzymes related to glutathione metabolism were normal or increased, consistent with the reticulocytosis. The concentrations of glycolytic intermediates, other than adenine nucleotides, were normal. The conversion of glucose, adenosine, and inosine to lactate was normal or increased. Autohemolysis was of the Dacie Type II. The concentrations of erythrocyte-reduced glutathione were high normal or elevated. The stained blood film showed a striking degree of basophilic stippling of the erythrocytes. Studies of the erythrocytes of the patient’s only known relative, a son, have failed to reveal any hematologic or enzymatic abnormalities. A direct causal relationship between RPK deficiency, high ATP concentrations, and nonspherocytic hemolytic anemia could not be derived from data now available. The final decision as to whether the deficiency is primary and causative or is an epiphenomenon requires investigation of additional cases.
Hemoglobins in Human Fetuses: Evidence for Adult Hemoglobin Production After the 11th Gestational Week
Authors: Pataryas Haris A. , Stamatoyannopoulos George 
Published: October 2019

Abstract
Hemoglobin patterns were examined in human fetuses in order to determine the onset of synthesis of adult hemoglobin in fetal red cells. In fetuses younger than 11 gestational wk, hemoglobin A could not be detected with electrophoretic techniques. A small amount of adult hemoblobin was constantly present, however, in hemolysates from fetuses older than 11 gestational wk, and it had the structural properties of adult hemoglobin A. Heterozygous fetuses had hemoglobins C and A or S and A as early as the 11th and 13th gestational wk. The findings are compatible with onset of beta hemoglobin gene action as early as the 11th gestational wk and synthesis of approximately 5% of adult hemoglobin by the end of the first and throughout the second trimester of gestation.
Studies of the Hemopoietic Microenvironment. I. Changes in the Microvascular System and Stroma During Erythropoietic Regeneration and Suppression in the Spleens of CF1 Mice
Authors: McCuskey Robert S. , Meineke Howard A. , Townsend Samuel F. 
Published: October 2019

Abstract
Specific alterations in the microvascular and connective tissue compartments of the hemopoietic microenvironment have been examined during erythropoietic regeneration and suppression in the murine spleen and bone marrow using in vivo microscopic and histochemical methods. The results have confirmed the concept of specific hemopoietic microenvironments and have demonstrated specific alterations in the microenvironment during erythropoietic stimulation and repression. Elevated erythropoiesis in the splenic red pulp is accompanied by an elevation in blood flow through the microvascular system. Both the linear velocity of flow and the number of sinusoids with blood flow in them increased significantly. In contrast, erythropoietic repression was accompanied by a decreased linear velocity of blood flow, as well as a marked increase in the amount of blood being stored in the splenic sinusoids. This also was the picture when diffuse granulopoiesis was present in the red pulp, or when granuloid or undifferentiated colonies were present. The chemical composition of the stroma in the spleen and bone marrow also varied during states of hemopoietic activity and, in addition, there were differences in the composition of the stroma between these two organs. In both organs, foci of early proliferating cells were enveloped by a coating of sulfated acid mucopolysaccharide. This coat persisted on cells in later stages of granulopoiesis but not on cells in the later stages of erythropoiesis. The latter were enveloped with a coating of neutral mucopolysaccharide. A tentative hypothesis to explain the mechanisms involved in producing these changes is discussed.
Studies of the Hemopoietic Microenvironment. II. Effect of Erythropoietin on the Splenic Microvasculature of Polycythemic CF1 Mice
Authors: McCuskey Robert S. , Meineke Howard A. , Kaplan Stephen M. 
Published: October 2019

Abstract
The effect of erythropoietin on the splenic microvascular system of polycythemic CF1 mice was studied using in vivo microscopic methods. Administration of a single dose (3 U) of erythropoietin resulted in an increase in the linear velocity of blood flow through the splenic sinusoids and a reduction in the number of sinusoids storing blood. This response was first seen 4-6 hr after injection; it persisted for 48 hr and was reduced markedly by 72 hr. By 120 hr the spleens were indistinguishable from controls. The response was specific for erythrogenic tissue, since no response was seen in the adjacent nonerythropoietic pancreatic tissue. The results suggest that the splenic microvascular response to erythropoietin may be indirect and may be mediated by the release of a vasoactive metabolite from the erythrogenic tissues surrounding the sinusoids. Erythropoietin-sensitive stem cells are suggested to be the source of such a metabolite.
Embryonic Origin of the Mouse Macrophage
Authors: Cline M. J. , Moore M. A. S. 
Published: October 2019

Abstract
Progenitor cells capable of giving rise to functional macrophages in in vitro culture were first detectable in the fetal yolk sac of the mouse between day 7 and 8 of gestation. Macrophage progenitors were not detectable outside the yolk sac until day 11. Although the early yolk sac contained macrophage precursors, no cells with the morphologic or functional characteristics of mouse promonocytes or more mature macrophages were observed. Promonocytes and macrophages were first identified in the 10-day yolk sac and 11-day fetal liver. These cells were characterized by surface receptors for IgG immunoglobulin, peroxidase activity (promonocytes), glass adherence, and phagocytosis of a large yeast particle (macrophages). From these observations, we conclude that the early fetal yolk sac is the embryonic site of origin of the macrophage precursor and that this precursor is "proximal" to the promonocyte on the pathway of sequential macrophage maturation.
Lymphocytotoxins and Pernicious Anemia
Authors: Goldberg Leonard S. , Cunningham Jo Ellen , Terasaki Paul I. 
Published: October 2019

Abstract
Sera from 60 patients with pernicious anemia were studied for the presence of lymphocytotoxins (LCT), blocking and binding autoantibodies to intrinsic factor, and gastric parietal cell autoantibody. LCT were found in 21 sera. Cytotoxic activity was detected at 15°C but not at 24°C and did not appear to have HL-A specificity. Autocytotoxins were present in four of eight patients tested. Blocking antibody to intrinsic factor was found in 34 sera, binding antibody in 14 sera, and parietal cell antibody in 48 sera. Sera from 14 patients contained all three types of autoantibodies, and 12 sera were void of these autoantibodies. Of the 14 sera with three types of autoantibodies, LCT were detected in ten; none of the 12 sera without autoantibodies showed cytotoxic activity. These studies suggest that LCT may reflect the degree of autoimmune derangement in pernicious anemia; alternatively, LCT may represent naturally occurring immunosuppressants.
RECENT STUDIES OF MULTIPLE MYELOMA: STERNAL AND RIB PUNCTURE AND THE RESULTS OF TREATMENT WITH STILBAMIDINE
Authors: PROPP SIMON , GORHAM L. WHITTINGTON , KANTOR SAMUEL 
Published: October 2019

Abstract
1. The value of bone marrow aspiration in the diagnosis of multiple myeloma was confirmed and discussed. This procedure should be utilized in all patients suspected of having this disease.
2. The importance of a reliable technic of studying bone marrow obtained by aspiration was stressed, and a method emphasizing certain important features described in detail.
3. The theory that multiple myeloma is derived from a dysplastic line of plasma cells originating in the bone marrow was supported by this study.
4. The original observation of Snapper has been confirmed, that after treatment of multiple myeloma patients with stilbamidine, large basophilic inclusion bodies can be demonstrated in the cytoplasm of a majority of myeloma cells obtained from bone marrow aspiration and stained by Wright’s stain. This was produced on a nonrestricted as well as on a low animal protein diet.
5. Relief of pain was produced in two out of five patients with multiple myeloma treated with stilbamidine. One patient who was relieved of pain was on a low animal protein diet while the diet of the second was unrestricted. In both caess basophilic inclusion bodies appeared in the myeloma cells following treatment. Stilbamidine therapy failed to allevaite pain or to produce basophilic granulation in the myeloma cells in three patients who exhibited hyperglobulinemia.
6. Relief of pain and vertigo occurred in one patient treated with stilbamidine while osteolytic lesions were observed to enlarge by roentgenological examination.
7. Trigeminal neuropathy with severe discomfort still continued six months following treatment in one patient.
8. An arrest or remission in the course of the disease was not obtained in five cases of multiple myeloma treated with stilbamidine.
THE OCCURRENCE OF THE PERIODIC ACID-SCHIFF REACTION IN VARIOUS NORMAL CELLS OF BLOOD AND CONNECTIVE TISSUE
Authors: WISLOCKI GEORGE B. , RHEINGOLD JACK J. , DEMPSEY EDWARD W. 
Published: October 2019

Abstract
An account is given of the periodic acid-Schiff reaction in the cytoplasm of various normal cells of blood and connective tissue of man, rhesus monkey and rabbit. Saliva treated control sections were used to distinguish glycogen from other reactive substances. The effects of malt diastase were compared briefly with those of saliva. The results of the present study may be summarized as follows (table 1):
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