bluuebunny/crossref_metadata_2025_split huggingface dataset:
Studies on Abnormal Hemoglobins
Authors: SINGER KARL , SINGER LILY 
Published: October 2019

Abstract
1. When sufficiently concentrated sickle cell hemoglobin containing solutions are exposed to a constant stream of CO2 gas, the hemolysates gel. This gelling phenomenon is indicative of the presence of S hemoglobin and cannot be obtained with any other type of human hemoglobin in the absence of S pigment. The lowest S hemoglobin concentration (Gm. per cent) of a hemolysate at which the gelling phenomenon can still be elicited is designated as its lowest gelling point.
2. A simple apparatus was developed to analyze the gelling phenomenon under standardized conditions. It could be shown that the lowest gelling points of hemolysates prepared from erythrocytes of the sickle cell trait (containing A + S hemoglobins), of the "C variant" (containing C + S hemoglobins), and from sickle cell anemia cells (containing S + F hemoglobins) differ distinctly. Further experiments suggest that the presence of A hemoglobin decreases the minimal amount of S pigment required for gel formation, and that type C hemoglobin reduces this amount even further. F hemoglobin seems to exert no significant influence on the gelling phenomenon. Serum albumin is also capable of decreasing the amount of S hemoglobin required for gelation.
3. A sickled erythrocyte is visualized as an S hemoglobin tactoid or gel, specifically influenced by the companion pigment which interacts with the S compound. Thus, in the sickle cell trait, a positive sickling test is not only caused by the presence of S hemoglobin, but also by its interaction with A hemoglobin. Only in the sickle cell anemia cells does sickling seem to depend solely upon the interaction of the S hemoglobin molecules.
4. The readily demonstrable differences of the lowest gelling points of hemolysates prepared from the various types of sickling red cells form the basis of the diagnostic gelling test which distinguishes sharply between sickle cell anemia and sickle cell trait erythrocytes. By this procedure atypical cases of sickle cell disease, for example, those whose erythrocytes contain C hemoglobin, may also be detected.
Studies in Rh Sensitization
Authors: WIENER ALEXANDER S. , NAPPI RAFFAELE , GORDON EVE B. 
Published: October 2019

Abstract
The distribution of the A-B-O blood groups among four hundred fifty-seven sensitized Rh negative mothers was compared with the distribution among eight hundred twenty-three nonsensitized Rh negative mothers. As expected, the frequencies of groups O and B were lower while that of group A was higher in the series of sensitized women. The difference in frequency of group A was 5 times its probable error and therefore statistically significant. Surprisingly, however, the difference proved to be due entirely to subgroup A1, while the frequency of subgroup A2 was the same in both series.
In a random series of four thousand nine hundred seventy-four Caucasoid individuals in New York City, the frequency of type rh’ was found to be 0.95 per cent. Statistical analysis of the Rh types of four hundred fifty-seven sensitized Rh negative mothers indicates, on the other hand, that the frequency of type rh’ is actually 0.41 per cent. The discrepancy is explained by postulating that in the control series, about half of the supposed type rh’ individuals were really type Rh1, since no special test had been made for Rh0 variants. In the series of sensitized Rh negative mothers, individuals of type Rh1 are automatically screened out since when factor Rh0 is present sensitization to the Rh0 factor is hardly likely to occur.
Humoral Regulation of Red Cell Production
Authors: ERSLEV ALLAN 
Published: October 2019

Abstract
Large amounts of plasma from rabbits, rendered anemic by bleeding, were injected into normal rabbits. A significant rise in the number of reticulocytes was observed in these rabbits. Control rabbits receiving the same amount of plasma from normal donor rabbits failed to show any significant change in the reticulocyte count. A definite increase in red blood cell count, hematocrit and per cent nucleated red cells of the bone marrow was noticed in 2 rabbits receiving repeated injections of plasma from anemic rabbits. It is concluded that plasma from rabbits rendered anemic by bleeding contains a factor capable of stimulating red cell production.
Immunologic Studies of Hemoglobins
Authors: CHERNOFF AMOZ I. 
Published: October 2019

Abstract
1. A quantitative immunologic technic for the determination of fetal hemoglobin has been developed using a specific anti fetal hemoglobin serum.
2. Fetal hemoglobin occurs in many normal adult individuals in extremely small quantities: values of up to 1.0 per cent of the total hemoglobin may be in the form of the embryonic compound.
3. The alkali-resistant hemoglobin fractions detected by chemical procedures in a number of hematologic diseases are, in all probability, identical with the normally occurring fetal hemoglobin of the red cells of newborn infants. It is suggested, therefore, that these diseases are characterized by abnormal quantities of fetal hemoglobin rather than by an abnormal fetal-like component.
Some Observations on Anemia in Rheumatoid Arthritis
Authors: JEFFREY M. R. 
Published: October 2019

Abstract
1. The results of investigations upon some aspects of anemia in rheumatoid arthritis are reported and discussed.
2. The anemia was essentially normocytic and hypochromic, the mean corpuscular volume being usually normal and the mean corpuscular hemoglobin content and concentration usually reduced. Anemia was more pronounced in the presence of very active disease.
3. Undue hemolysis was not of importance.
4. The myelogram showed no gross abnormality but suggested impaired maturation of normoblasts and retarded hemoglobin formation.
5. The blood and plasma volumes were not essentially abnormal, though the frequent asthenic habitus and loss of weight might produce apparently exalted values in terms of body weight. In the more anemic cases, changes common to many anemias were found, viz., normal plasma volume with decreased corpuscle and whole blood volume.
6. The plasma iron concentration was usually reduced; subnormal values were invariable in cases with very active disease or marked anemia.
7. The iron-binding capacity of the serum might be raised by iron deficiency or slightly reduced but not enough to be a factor in producing or maintaining anemia.
8. As judged by changes in the plasma iron, intestinal absorption of iron after a single maximal therapeutic dose was variable. Some cases showed the excellent absorption and therapeutic response characteristic of simple iron deficiency. Others gave strong presumptive evidence of impaired absorption.
Control of the Postirradiation Hemorrhagic State by Platelet Transfusions
Authors: WOODS M. C. , GAMBLE FRANCES N. , FURTH J. , BIGELOW R. R. 
Published: October 2019

Abstract
Suspensions of concentrated, homologous blood platelets when introduced intravenously into lymph-cannulated dogs and rats (nine to twelve days after exposure to an LD50 or greater dose of x-rays) have been shown to eliminate the large scale diversion of erythrocytes into the lymph during the hemorrhagic phase of radiation injury. By means of injection of platelets preinjection levels of below 50,000 were raised to an average of 107,000 in 6 dogs, and to an average of 310,000 in five rats at 15 to 30 minutes after injection. All platelet-injected animals showed clearing of the bloody lymph within the first hour after injection, and within 5 to 6 hours the average erythrocyte count in the dogs dropped from an average of 270,000 to an average of 12,000 and in the rats from an average of 900,000 to an average of 30,000. No depression of lymph erythrocyte counts was found in control rats receiving plasma only. Lymph erythrocyte counts in the platelet recipients remained low for 24 to 36 hours after injection; bleeding into the lymph space reappeared in both rats and dogs when the platelet level dropped below approximately 50,000. Blood platelet and lymph erythrocyte levels are excellent indices to quantitate the severity of the hemorrhagic phase of radiation injury and to gauge the results of therapeutic procedures.
While the mechanism of platelet action remains to be demonstrated, the present studies support the thesis of earlier workers that platelets are essential to maintain vascular integrity and that a platelet deficiency is the main cause of the postirradiation hemorrhagic state.
Proteolytic and Fibrinolytic Activity of Serum
Authors: CLIFFTON EUGENE E. , CANNAMELA DOLLY A. 
Published: October 2019

Abstract
1. The fibrinolytic and proteolytic activity of serum plasminogen, activated by Streptokinase and Staphylokinase and measured by clot lysis and casein digestion, was studied in the following species of animals: human, cow, dog, horse, monkey, pig, rabbit, rat, and chicken.
2. A significant difference in fibrinolytic and proteolytic activity of serum plasminogen (activated by staphylokinase) was observed in three animal species: monkey, horse, and human. The significance of this observation is briefly discussed.
Hemolytic Disease of the Newborn Infant Caused by Maternal Sensitization to the Blood Factor hr' (c)
Authors: GRUNDORFER JOSEPH 
Published: October 2019

Abstract
1. Intragroup Rh incompatibility between an Rh-positive mother and an Rh-positive infant presents an unusual pattern of maternal isosensitization.
2. The antibody found in the sera of two Rh-positive mothers appeared to be of anti-hr' (anti-c) specificity.
3. Evidence of hemolytic disease in both infants was found and the findings directly correlated with this maternal antibody.
4. The welfare of the infant greatly depends on prompt recognition of this unusual pattern of maternal isosensitization and proper treatment of its disease.
5. An attempt has been made to evaluate the role of multiple transfusions of Rh-negative blood which had been given to an Rh-positive mother four years prior to gestation.
6. The wide spacing of several transfusions or transfusion and pregnancy respectively appears to be probably the most important factor in the development of isosensitization.
7. The possible danger in using Rh-negative blood as a universal safe donor is briefly discussed.
Paroxysmal Nocturnal Hemoglobinuria
Authors: CROSBY WILLIAM H. 
Published: October 2019

Abstract
1. Paroxysmal noctural hemoglobinuria is believed to be an acquired disease of the hematopoietic system in which abnormal red cells, white cells, and platelets are produced. The lesion of the cells probably involves the stromal proteins in such a fashion that they are susceptible to the proteolytic effect of a sytem of normal plasma enzymes. The severity of the disease depends upon the degree of sensitivity of a given patient’s blood cells to the plasma hemolytic system.
2. The plasma enzyme system that mediates the destruction of PNH cells consists of hemobytic factors and their inhibitors. The most important inhibitor is easily destroyed by thrombin, allowing increased hemolysis. As a consequence of this, any reaction in the patient that involves the blood coagulation system is apt to cause a hemolytic crisis.
3. PNH red cells are destroyed intravascularly causing hemoglobinemia. Hemoglobinuria, hemosiderinuria, and abnormalities of iron metabolism are sequels of this reaction.
4. Anemia in PNH is a result of hemolytic disease (short red cell life span) together with a relative bone marrow deficiency.
5. Hemolytic crises in PNH occur when plasma hemolytic activity increases. The intensity of activity depends upon a balance that exists between the hemolytic enzymes in the patient’s plasma and two inhibitory factors. Hemolytic crises take place when the balance is disturbed in favor of the hemolytic factors. Increased hemolysis at night may be due to changes in the balance of the inhibitor-hemolysin system in addition to the effect on pH that may be produced by retention of CO2 during sleep. Hemolytic crises have sometimes been due to the temporary appearance of an autoimmune reaction.
6. Aregenerative crises occur when the bone marrow temporarily suspends production of blood cells. This may result in severe anemia, agranulocytic angina, or both. Purpura is rare in PNH.
7. Chronic leukopenia in PNH is believed to be due to a leukocytic abnormality. The susceptibility to infection of patients with PNH may be related to the defective white cells.
8. Thrombocytopenia is believed to be due to a platelet abnormality. The platelets are prone to agglutinate, and their abnormality may be the basis of the susceptibility to thrombosis that these patients exhibit.
9. The sensitivity of the platelets to the PNH hemolytic enzymes and the sensitivity of the PNH inhibitor to the action of thrombin suggests that a vicious cycle of activity exists between the PNH hemolytic system and the coagulation system of the blood.
10. Dicumarol is able to impede this cycle. Its use has sometimes relieved the anemia. Dicumarol is of special value in protecting patients with PNH against thrombotic accidents. Heparin, on the other hand, by acting against the PNH inhibitor system actually causes increased hemolysis.
11. Etiologic factors are discussed, but the etiology of PNH is unknown.
12. As of July 1953, there had been published at least one hundred and sixty-two case reports of PNH.
The Immunologic Specificity of Antiserum for Trypsin-Treated Red Blood Cells and Its Reactions with Normal and Hemolytic Anemia Cells
Authors: Dodd Matthew C. , Wright Claude-Starr , Baxter J. Albert , Bouroncle Bertha A. , Bunner Alvinza E. , Winn Henry J. 
Published: October 2019

Abstract
The Addendum which appeared on page 647 of an article entitled "The Immunologic Specificity of Antiserum for Trypsin-Treated Red Blood Cells and Its Reactions with Normal and Hemolytic Anemia Cells" by Matthew C. Dodd, Claude-Starr Wright, J. Albert Baxter, Bertha A. Bouroncle, Alvinza E. Bunner, and Henry J. Winn (Blood 8: 640-647, 1953) belongs, instead, with the article entitled "Production of Nucleophagocytosis by Rabbit Antileukocytic Serum" by Hyman J. Zimmerman, John R. Walsh, and Paul Heller (Blood 8: 651-654, 1953).
A mutation of the common receptor subunit for interleukin-3 (IL-3), granulocyte-macrophage colony-stimulating factor, and IL-5 that leads to ligand independence and tumorigenicity
Authors: D'Andrea R , Rayner J , Moretti P , Lopez A , Goodall GJ , Gonda TJ , Vadas M 
Published: October 2019

Abstract
The cytokines interleukin-3, interleukin-5, and granulocyte-macrophage colony-stimulating factor bind with high affinity to a receptor complex that contains a ligand-specific alpha-chain and a common beta-chain, h beta c. We report here the isolation of a mutant form of h beta c, from growth factor-independent cells, that arose spontaneously after infection of a murine factor-dependent hematopoietic cell line (FDC-P1) with a retroviral h beta c expression construct. Analysis of this h beta c mutation shows that a small (37 amino acid) duplication of extracellular sequence that includes two conserved sequence motifs is sufficient to confer ligand-independent growth on these cells and lead to tumourigenicity. Because this is a conserved region in the cytokine receptor superfamily, our results suggest that the large family of cytokine receptors has the capacity to become oncogenically active.
Roles for integrin very late activation antigen-4 in stroma-dependent erythropoiesis
Authors: Yanai N , Sekine C , Yagita H , Obinata M 
Published: October 2019

Abstract
Adhesion molecules are required for development of hematopoietic stem and progenitor cells in the respective hematopoietic microenvironments. We previously showed that development of the erythroid progenitor cells is dependent on their direct adhesion to the stroma cells established from the erythropoietic organs. In this stroma-dependent erythropoiesis, we examined the role of adhesion molecules in erythropoiesis by blocking antibodies. The development of the erythroid cells on stroma cells was inhibited by anti-very late activation antigen-4 (VLA-4 integrin) antibody, but not by anti-VLA-5 antibody, although the erythroid cells express both VLA-4 and VLA-5. Whereas high levels of expression of vascular cell adhesion molecule-1 (VCAM-1) and fibronectin, ligands for VLA-4, were detected in the stroma cells, the adhesion and development of the erythroid progenitor cells were partly inhibited by the blocking antibody against VCAM-1. VLA-5 and fibronectin could mediate adhesion of the erythroid progenitor cells to the stromal cells, but the adhesion itself may not be sufficient for the stroma-supported erythropoiesis. The stromal cells may support erythroid development by the adhesion through a new ligand molecule(s) for VLA-4 in addition to VCAM-1, and such collaborative interaction may provide adequate signaling for the erythroid progenitor cells in the erythropoietic microenvironment.
Effect of granulocyte-macrophage colony-stimulating factor on sepsis- induced organ injury in rats
Authors: Toda H , Murata A , Oka Y , Uda K , Tanaka N , Ohashi I , Mori T , Matsuura N 
Published: October 2019

Abstract
The administration of granulocyte-macrophage colony-stimulating factor (GM-CSF) to patients with severe active infections has been questioned because activation of neutrophils may cause tissue injury. To identify the effect of GM-CSF administration on severe sepsis, we examined the survival rate and pathologic changes in vital organs using the rat lethal sepsis model. Rats received 20 micrograms of recombinant murine GM-CSF (rmGM-CSF) 3 hours after the onset of peritonitis induced by cecal ligation and puncture. After 48 hours, the survival rate did not improve, and earlier deaths than in the control group were observed. In addition, the inhibition of early leuko-sequestration in the peritoneal cavity was seen in animals treated with GM-CSF. These results suggested that the administration of rmGM-CSF after the onset of sepsis was not beneficial; thus, we concluded that care should be taken in the clinical use of GM-CSF in severe infection.
A reverse transcriptase-polymerase chain reaction detects heterogeneous chimeric mRNAs in leukemias with 11q23 abnormalities
Authors: Yamamoto K , Seto M , Iida S , Komatsu H , Kamada N , Kojima S , Kodera Y , Nakazawa S , Saito H , Takahashi T 
Published: October 2019

Abstract
The MLL gene involved in 11q23 translocations found in the majority of infantile leukemias and some secondary leukemias makes fusion transcripts with genes such as LTG4 (chromosome 4), LTG9 (chromosome 9), and LTG19 (chromosome 19) as a result of reciprocal translocation. We have examined 25 cases of leukemias with 11q23 abnormalities by Southern blot analysis and the reverse transcriptase-polymerase chain reaction (RT-PCR). Using various primer pairs, chimeric mRNAs could be amplified in 6 of 7 leukemias with t(4;11), 6 of 8 leukemias with t(9;11) including secondary leukemia, 8 of 9 leukemias with t(11;19), and 1 with a deletion at 11q23. The chimeric mRNAs were heterogeneous and differential usage of the MLL exons was found, irrespective of the partner chromosomes. Sensitivity studies showed that a single clone with chimeric mRNA in 10(4) to 10(5) cells could be detected. These findings show that the present RT-PCR settings provide a rapid, accurate, and sensitive tool for diagnosing leukemias with 11q23 translocations and for monitoring response to therapy in these patients.
Interstitial insertion of retinoic acid receptor-alpha gene in acute promyelocytic leukemia with normal chromosomes 15 and 17
Authors: Hiorns LR , Min T , Swansbury GJ , Zelent A , Dyer MJ , Catovsky D 
Published: October 2019

Abstract
The translocation t(15;17)(q22;q21) is seen exclusively in patients with acute promyelocytic leukemia (APL) and in the promyelocytic blast crisis of chronic myeloid leukemia (CML). This translocation juxta- poses the promyelocytic leukemia (PML) gene on chromosome 15 and the retinoic acid receptor-alpha (RARA) gene on chromosome 17, resulting in the formation of a chimeric mRNA transcript. We describe a patient with the microgranular variant form of APL, with no detectable cytogenetic abnormality of either chromosomes 15 or 17, who nevertheless had juxtaposition of PML and RARA genes and expressed a chimeric transcript. Conventional cytogenetics showed the karyotype 46,XY,d- er(3)t(3;8)(p25;q12). Fluorescent in situ hybridization (FISH) with paints for chromosomes 8, 15, and 17 confirmed the presence of structurally intact chromosomes 15 and 17 and trisomy for chromosome 8q. Nevertheless, FISH using cosmid probes for PML and RARA showed their juxtaposition on one chromosome 15 homolog. Both genes were also present on their normal homologs; in addition, part of the RARA gene was still present on the remaining chromosome 17. DNA analysis by Southern blotting, performed with a variety of probes including PML, RARA and retinoic acid receptor-beta (RARB), showed a rearrangement in PML. Reverse transcriptase polymerase chain reaction (RT-PCR) confirmed the existence of hybrid transcripts of 276, 455 bp and 623 bp, from PML- RARA on the der(15) chromosome, consistent with alternate exon splicing of the long form of the transcript occurring in 50% to 60% of patients with APL. Our results show that APL patients with cytogenetically normal chromosomes 15 and 17 may, nevertheless, have involvement of both PML and RARA genes defining a subgroup of APL, t(15;17)- negative/PML-RARA-positive which is analogous to Philadelphia chromosome-negative/BCR-ABL-positive CML. In this case, the presence of chimeric transcripts suggests that treatment with all-trans RA may be warranted in APL, even in the absence of detectable cytogenetic change, showing the usefulness of RT-PCR or FISH to aid diagnosis.
The heterogeneity of azurophil granules in neutrophil promyelocytes: immunogold localization of myeloperoxidase, cathepsin G, elastase, proteinase 3, and bactericidal/permeability increasing protein
Authors: Egesten A , Breton-Gorius J , Guichard J , Gullberg U , Olsson I 
Published: October 2019

Abstract
Azurophil granules of myeloid cells form in promyelocytes. They store cytotoxic and digestive agents which when released are involved in the defense against infection. In order to characterize the intragranular distribution of these agents, ultrastructural methods using immunogold were used on promyelocytes. Azurophil granules were divided into nucleated, large spherical (large azurophil) and small electron-dense (small azurophil) granules. Myeloperoxidase showed a peripheral distribution of large azurophils and a uniform distribution of small and nucleated azurophils, consistent with previous findings. Likewise, the major neutral proteases of azurophils, cathepsin G, granulocyte elastase, and proteinase 3, displayed a similar distribution, with a peripheral localization in large azurophils and a uniform distribution in small and nucleated azurophils, except for proteinase 3, which was associated with the crystalloid structure in nucleated azurophils. In contrast, the bactericidal/permeability increasing protein, which is bacteristatic and bactericidal for Gram-negative bacteria, was localized to the membrane area in all types of azurophil granules, consistent with a suggested association of this protein with the granule membrane. The observed differences in intragranular distribution of the proteins investigated may reflect variations in binding to matrix structures and granule membranes.