bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cytofluorometric analysis of the kinetics of lymphocyte transformation after phytohemagglutinin stimulation: comparison with the kinetics of thymidine incorporation
Authors: Utsinger PD , Yount WJ , Fallon JG , Logue MJ , Fuller CR , Elliott D 
Published: October 2019

Abstract
The technique of flow cytofluorometry has been employed to assess the response of unfractionated and highly purified human lymphocyte subpopulations to phytohemagglutinin (PHA) and pokeweed mitogen. Normal values for cytofluorometric responses were established and compared to the uptake of tritiated thymidine in simultaneous experiments. Cytofluorometric analysis offered the advantages of increased sensitivity and direct measurement of DNA content per cell, and provided percentages and absolute numbers of responding cells. B-cell responses to pokeweed mitogen were absent, but brisk T-cell responses were noted. Between 4% and 8% of highly purified human B cells were found to respond to PHA by increasing their DNA content; modest but significant uptake of tritiated thymidine by B cells following PHA stimulation was also observed.
Enhancement of colony-stimulating activity production by lithium
Authors: Harker WG , Rothstein G , Clarkson D , Athens JW , Macfarlane JL 
Published: October 2019

Abstract
Since lithium causes granulocytosis in some patients, its effect upon granulocyte production was investigated using mouse marrow in the agar culture system. When lithium was added to semisolid cultures of mouse marrow, there was no stimulation of colony formation in the absence of colony-stimulating activity (CSA). In addition, lithium did not potentiate the action of already formed CSA. However, lithium did stimulate the production of CSA by lung tissue. Lithium enhancement of CSA production was blocked by puromycin, indicating that lithium action required active new protein synthesis. It was concluded that lithium promoted enhanced granulocyte production in vitro by stimulating the synthesis of CSA.
T and B lymphocyte antigen-positive null cell leukemias
Authors: Kaplan J , Ravindranath Y , Peterson WD Jr 
Published: October 2019

Abstract
To clarify the nature of null cell acute lymphoblastic leukemia (ALL) we tested null lymphoblasts and other leukemic cells for human T and B lymphocyte antigens detected by reciprocally absorbed rabbit antisera prepared against autologous T and B lymphoblast cell lines HSB-2 and SB. Blasts from 4 of 4 patients with T cell leukemia and from 2 of 10 patients with null cell leukemia expressed T cell but not B cell antigens. Blasts from 8 of 10 patients with null cell leukemia, one patient with B cell leukemia, and 3 of 3 patients with acute myelogenous leukemia expressed B cell but not T cell antigens. T lymphocyte antigen-positive null cell ALL appear to be a form of T cell ALL. B antigen-positive null cell leukemia could arise from either immature B cell precursors or from multipotential stem cells.
Semicontinuous flow centrifugation for the pheresis of immunocompetent cells and stem cells
Authors: Weiner RS , Richman CM , Yankee RA 
Published: October 2019

Abstract
Mononuclear cells from human peripheral blood were purified by semicontinuous flow centrifugation (SCFC) using the Haemonetics model 30 blood cell separator; 64% +/- 7% of the mononuclear cells in 600 ml of peripheral blood were collected in a 30-ml volume. Analysis of sequential 5-ml aliquots of the mononuclear cell concentrate revealed that both immunocompetent cells and granulocytic progenitor cells (CFU- C) were proportional to the cell count throughout the buffy coat. In vitro pheresis of large volumes of human bone marrow resulted in recovery of 63% of the cells, 12% of the hemoglobin, and 84% of the CFU- C in 20% of the original volume. Further centrifugation eliminated 80% of the platelets without loss of cells or CFU-C. SCFC of peripheral blood or bone marrow selectively concentrated mononuclear cells and reduced the contamination by granulocytes and erythrocytes. Large numbers of mononuclear cells can thus be collected for studies in vitro or for cryopreservation and the autologous reconstitution of immunosuppressed or myelosuppressed patients undergoing intensive antitumor therapy.
In vitro stimulation of primate hemoglobin synthesis by L-thyroxine
Authors: Fuhr JE , Gengozian N , Overton M 
Published: October 2019

Abstract
Bone marrow cells from adult and abortus primates (marmosets) were incubated in vitro to determine their responsiveness to L-thyroxine. 3H- leucine incorporation into purified globin chains was the parameter assayed to determine responsiveness. Bone marrow from spontaneously aborted animals consistently was stimulated by the presence of physiologic levels of L-thyroxine. Bone marrow cells from adult animals were unaffected by the hormone.
Congenital neutropenia: an intrinsic cell defect demonstrated by electron microscopy of soft agar colonies
Authors: Zucker-Franklin D , L'Esperance P , Good RA 
Published: October 2019

Abstract
Congenital neutropenia (CN), a disease characterized by recurrent infections leading to death in infancy, shows a maturation arrest of the myeloid series at the promyelocyte-myelocyte level. The potential value of marrow transplantation in this disease would be determined by the nature of the underlying defect. However, studies to date have failed to define whether the defect is intrinsic in the cells or attributable to “environmental” factors. Therefore, marrow of four patients with CN was cultured on soft agar, and the colonies were analyzed by a newly developed ultrastructural method. In parallel, patients' cells were used in feeder layers for normal marrow. Although the patients' colonies appeared grossly normal in size and number, electron microscopy showed only rare neutrophil colonies. These colonies contained markedly aberrant cells exhibiting asynchronous nucleocytoplasmic maturation, convoluted nuclei, excessive cytoplasm, and dearth of granules. Monocyte and eosinophil colonies differentiated normally. Patients' cells and sera supported growth of normal colonies. The studies have demonstrated unequivocally that the neutrophil cell line of patients with CN is intrinsically defective and suggest that attempts at marrow grafting are warranted.
Effect of cyanide on NADPH oxidation by granules from human polymorphonuclear leukocytes
Authors: DeChatelet LR , McPhail LC , Shirley PS 
Published: October 2019

Abstract
Cyanide has been shown to stimulate both oxygen uptake and hexose monophosphate shunt activity in phagocytizing human polymorphonuclear leukocytes. It also stimulates the oxidation of NADPH by a particulate fraction derived from phagocytizing cells. This stimulation of NADPH oxidase is not observed in the presence of exogenous Mn2+. Studies with purified enzymes have shown that CN- also stimulates NADPH oxidation by horseradish peroxidase or lactoperoxidase, suggesting that the respiratory burst might be initiated by activation of a peroxidase-like enzyme in the human polymorphonuclear leukocyte. Based on studies of others, however, it does not appear as though the enzyme is identical to myeloperoxidase. The mechanism of the CN- stimulation appears to involve an oxidatic chain reaction, since it stimulates markedly NADPH oxidation in the presence of an artificial superoxide-generating system.
Gamma heavy chain disease: clinical aspects and characterization of a deleted, noncovalently linked gamma1 heavy chain dimer (BAZ)
Authors: Faguet GB , Barton BP , Smith LL , Garver FA 
Published: October 2019

Abstract
This report describes the clinical and immunoglobulin features of a patient with gamma heavy chain disease (HCD), who presented with a clinical picture suggestive of an underlying malignancy rather than the usual picture of lymphoma or granulomatous disease. A unique clinical feature was the nearly total replacement of the submaxillary glands by plasma cells. The patient's serum and urine contained a paraprotein, gammaHCD protein BAZ, which belongs to the gamma1 subclass and forms noncovalently linked dimers with a molecular weight of approximately 60,000 daltons. This mutant protein exhibited a deletion which encompassed most of the variable (V) region, the first constant domain (CH 1), and the hinge region. In addition, preliminary structural analyses demonstrated the replacement of alanine by glycine in position 431 of the carboxyterminal octadecapeptide. This substitution may possibly represent another allotypic marker on IgG1 proteins.
Stimulation by human placental conditioned medium of hemopoietic colony formation by human marrow cells
Authors: Burgess AW , Wilson EM , Metcalf D 
Published: October 2019

Abstract
Medium conditioned by human placental tissue was found to stimulate granulocytic and monocytic colony formation by human marrow cells in semisolid agar cultures. The colony-stimulating activity of unfractionated conditioned medium was equivalent to the activity of standard peripheral blood underlayers. Placentas were a reliable source of active material, and one placenta provided enough material to stimulate 5,000–10,000 cultures of normal or leukemic cells. The colony- stimulating factor in human placental conditioned medium (CSFHPCM) was concentrated and purified 1800-fold using ammonium sulfate precipitation, calcium phosphate gel absorption, DEAE-cellulose batch absorption, gel filtration on Sephadex G-150, and polyarcylamide gel gel electrophoresis. The active factor behaved on gel filtration as a macromolecule with an apparent molecular weight of 30,000 daltons. The active factor in placental conditioned medium was not dependent on the presence of adherent marrow cells with endogenous colony-stimulating activity.
cGMP stimulation of stem cell proliferation
Authors: Oshita AK , Rothstein G , Lonngi G 
Published: October 2019

Abstract
The process by which resting hemopoietic stem cells become activated is poorly understood, but it has been suggested that cyclic nucleotide levels in the cell may play an important role. In the present study, the effect of various, nucleotides and stimulators of nucleotide synthesis upon the formation of in vitro granulocyte colonies has been examined. It was found that physiologic concentrations of 3′, 5′-cyclic guanosine monophosphate enhanced the formation of granulocyte- macrophage colonies in the presence of colony-stimulating activity. The cells sensitive to cGMP activation were resistant to thymidine suicide and could not be activated by colony-stimulating activity alone. Therefore it was suggested that the cGMP sensitive stem cell was an ordinarily resting stem cell which was triggered into a proliferative state by cGMP.
Carriers with excessively low factor VIII procoagulant activity (VIII AHF): a study of two unrelated families with mild hemophilia A
Authors: Eyster ME , Ladda RL , Bowman HS 
Published: October 2019

Abstract
Two unrelated families are described with mild hemophilia A in whom six obligate carriers had unusually low VIII AHF levels. In each family, successive generations of males were affected with hemophilia A as determined by low VIII AHF in the presence of normal VIII AGN and VIII VWF levels. In the first family, two of five obligate carriers had low VIII AHF levels associated with clinical bleeding and one other had a history of bleeding. While receiving oral contraceptives, one of these two carriers was found to have a normal VIII AHF level. In the second family, four cousins below age 10 who were obligate carriers had significantly low VIII AHF levels, while a paternal aunt and paternal grandmother who were also obligate carriers had VIII AHF levels within the normal range. Hemorrhagic diathesis in multiple obligate carriers in these families is not readily explained by the Lyon hypothesis, and suggests that these families may be exmaples of an unusual allelic form of hemophilia A or that they may be transmitting several independent genes affecting VIII AHF levels. Our experience suggests that VIII AHF levels should be determined on all obligate or possible carriers prior to surgery to identify those individuals at risk for postoperative bleeding. Furthermore, it is suggested that hormonal therapy might be effective in the management of carriers with low levels of VIII AHF and clinical bleeding.
Demonstration and mode of action of an inhibitor for activated Hageman factor (factor XIIa) of the intrinsic blood coagulation pathway from Schistosoma mansoni
Authors: Tsang VC , Damian RT 
Published: October 2019

Abstract
An anticoagulant activity from adult Schistosoma mansoni whole worm homogenate is described. The inhibitor appears to be specific for the contact activation step of the intrinsic pathway. Experiments with both human and mouse plasmas have defined the specificity of the inhibitor as follows: (1) It lengthens the partial thromboplastin time of normal plasma. (2) It has no effect on the prothombin time and Russell's viper venom time of normal plasma. (3) Preactivation of normal plasma by a contact activator such as Celite eliminates essentially all inhibitory activity. (4) The inhibitor appears to be heat stable and can be precipitated by centrifugation above 27,000 g. (5) The inhibitor has no effect on the activation of factor XII by Celite. (6) The activation of factor XI by factor XIIa, however, is inhibited by the schistosomal inhibitor. The above data are consistent with the view that S. mansoni adults possess an anticoagulant that is capable of specifically inhibiting the conversion of factor XI to factor XIa by factor XIIa.
Variations in lymphocyte counts four hours after administration of hydrocortisone in patients with chronic lymphocytic leukemia
Authors: Dighiero G , Vaugier G , Charron D , d'Athis P , Binet JL 
Published: October 2019

Abstract
The peripheral lymphocyte count and the number of large unstained cells (LUC) were investigated prior to and 4 hr after a single intravenous injection of 400 mg of hydrocortisone in 23 controls and 51 patients with lymphoid disorders (43 chronic lymphocytic leukemia, 3 cases of Waldenstrom macroglobulinemia, 2 hairy cell leukemias, 1 Sezary syndrome, and 2 cases of infectious mononucleosis). A reduction in both the peripheral lymphocyte counts and the number of LUC was observed in all normal controls, the mean decrease being 54% and greater than 60%, respectively, with differences according to age. In chronic lymphocytic leukemia (CLL), the peripheral lymphocyte count showed a variable response: decrease, no change, or increase. A correlation was shown to exist between a decrease in peripheral lymphocyte counts and anatomical- clinical staging: patients with involvement restricted to blood and bone marrow very often exhibited a drop in their peripheral lymphocyte count (p less than 0.01). In addition, the percentage of circulating T lymphocytes was higher (54%) in CLL patients whose peripheral lymphocyte count dropped than in other CLL patients (p less than 0.001).
Chronic myeloid leukemia: physical and functional similarities to acute leukemia
Authors: Senn JS , Price GB 
Published: October 2019

Abstract
Blast crisis, closely resembling acute leukemia, is the usual terminal event in chronic myeloid leukemia. Using physical (“fingerprint”) and cultural (colony-forming) methods, we have demonstrated distinctive patterns in the stable phase of chronic myeloid leukemia and in blast crisis. An unusual fingerprint alteration preceding the onset of the terminal phase is noted, and cell culture perturbation is evident at different stages of the disease. Our findings indicate that the application of these methods to the study of hemopoietic disorders is valid, and suggest that the use of such techniques may allow a better understanding of the complex cellular events occurring in the course of chronic myeloid leukemia.
Induction of serum stimulation and plasma cell proliferation during chemotherapy of multiple myeloma
Authors: Karp JE , Burke PJ , Humphrey RL 
Published: October 2019

Abstract
Sequential sera from 45 patients with multiple myeloma (MM) and from 6 patients with solid tumors but normal bone marrows who received cyclophosphamide, 15 mg/kg/day for 4 days, were assayed for their effects on tritiated thymidine (3H-TdR) incorporation by normal bone marrow cells and malignant plasma cells. Pretreatment sera from 23 of the 45 patients with MM inhibited normal marrow cell proliferation relative to the effects of normal sera. Of these 45 sera, 30 inhibited plasma cell proliferation. This humoral inhibition was overcome by the induction of humoral stimulation at a predictable time during chemotherapy. The sera obtained sequentially from patients with MM and patients with normal bone marrows increased 3H-TdR uptake by both cell types by days 12–15 of therapy. Sequential changes in malignant marrow plasma cell 3H-TdR labeling indices paralleled the changes in serum activity, with an increased tumor cell growth fraction occurring at the time of peak serum stimulatory activity. The relationship between serum stimulation and malignant plasma cell proliferation was confirmed in vitro.
Chronic granulocytic leukemia: cytogenetic conversion of the bone marrow with cycle-specific chemotherapy
Authors: Smalley RV , Vogel J , Huguley CM Jr , Miller D 
Published: October 2019

Abstract
Sixteen patients with Ph1-positive chronic granulocytic leukemic (CGL) were entered on a pulsing chemotherapy program consisting of cytosine arabinoside 100 mg/sq m/day X 5 and thioguanine 100 mq/sq m/day X 5 every 21 days in an attempt to convert the Ph1-positive marrow to a Ph1- negative state and thereby achieve a complete remission. Twelve patients had an adequate trail of drug treatment, and ten of these had adequate chromosome examinations. There were two “conversions,” one of which was maintained for 5+ mo, while the other was transient. The program was unacceptable, however, to most patients due to intolerable nausea and vomiting. Thus a prospective chemotherapeutic attempt to convert a Ph1-positive marrow without splenectomy has induced a conversion in two of ten patients. Other regimens which might induce less nausea and vomiting and a higher rate of conversions should be sought in future attempts to alter the invariably fatal outcome of CGL.
Intracellular enzymes of collagen biosynthesis in human platelets
Authors: Anttinen H , Tuderman L , Oikarinen A , Kivirikko KI 
Published: October 2019

Abstract
Activities of four intracellular enzymes of collagen biosynthesis-- prolyl hydroxylase, lysyl hydroxylase, collagen galactosyltransferase, and collagen glucosyltransferase--were demonstrated in human platelets, and the presence of prolyl hydroxylase protein was further demonstrated by direct radioimmunoassay. The ratio of the specific activities of the four enzymes in the four enzymes in the human platelet extract to those in human adult skin extract varied from about 0.1 to 1, the lowest relative activity being found with prolyl hydroxylase and the highest with collagen glucosyltransferase. Only a very small amount of prolyl hydroxylase protein, probably 1%, was in the form of the active enzyme tetramer. The collagen glucosyltransferase from human platelets readily glucosylated galactosylhydroxylysine in denatured collagen, but did not glucosylate native collagen. Also, native collagen did not act as an inhibitor of the glucosylation reaction. Therefore, platelet collagen glucosyltransferase cannot form either an enzyme--substrate complex or an enzyme--inhibitor complex with native collagen. The results thus argue against the theory which maintains that platelet collagen glucosyltransferase is involved in collagen--platelet adhesion.
Myeloperoxidase--H2O2--halide system: cytotoxic effect on human blood leukocytes
Authors: Clark RA , Klebanoff SJ 
Published: October 2019

Abstract
Myeloperoxidase, H2O2, and a halide form a potent antimicrobial and cytotoxic system of the polymorphonuclear leukocyte. A cytotoxic effect of this system on human blood leukocytes is demonstrated, employing 51Cr release and dye exclusion assays. Cytotoxicity is dependent on enzymatically active myeloperoxidase, H2O2, or a peroxide-generating enzyme system and either chloride or iodide. Cell damage is rapid, with maximal levels of 51Cr release occurring within 30--60 min. Approximately equal sensitivity to the peroxidase system is observed for polymorphonuclear leukocytes and mononuclear leukocytes. Since myeloperoxidase and H2O2 are released from polymorphonuclear leukocytes under certain conditions, such as during particle ingestion, it is suggested that peroxidase-mediated leukocyte injury may be an important feature of the inflammatory response.
ABO blood group system and bone marrow transplantation
Authors: Gale RP , Feig S , Ho W , Falk P , Rippee C , Sparkes R 
Published: October 2019

Abstract
The role of the ABO blood group system in determining the outcome of bone marrow transplantation was investigated in 53 patients with aplastic anemia and acute leukemia grafted from HLA-identical siblings. There was no correlation between ABO compatibility and marrow engraftment, graft rejection, or graft-versus-host disease. In 5 recipients with antibodies prior to transplantation to antigens of the ABH system present on the cells of their donors, plasma exchange and antibody absorption in vivo were effective in permitting engraftment of ABO-incompatible bone marrow. These findings indicate that the ABO system is not a clinically significant barrier to successful bone marrow transplantation in otherwise histocompatible individuals.
An unusual case of leukemia with high fetal hemoglobin: demonstration of abnormal hemoglobin synthesis localized in a red cell clone
Authors: Pagnier J , Lopez M , Mathiot C , Habibi B , Zamet P , Varet B , Labie D 
Published: October 2019

Abstract
A high level of fetal hemoglobin was found in an 8-yr-old boy without any hematologic disorders except for a moderate anemia. The absence of hemoglobin abnormalities in the parents led us to suspect a latent malignant disease that, on follow-up, was confirmed to be myelomonocytic leukemia. Hemoglobin biosynthetic studies provided evidence of unbalanced synthesis of globin subunits by reticulocytes, while the production of non-alpha chains was equal to that of alpha chains in bone marrow cells. The expression of red cell antigen i was increased, while those of I, A, and A1 antigens were found to decrease progressively. Two populations of erythrocytes, A-positive and A- negative, were distinguished and could be separated by differential agglutination. Unbalanced globin chain synthesis, increased fetal hemoglobin, and antigenic changes of the membrane were shown to be restricted to the A-negative population. The biologic data were not entirely consistent with a genuine reversion to fetal erythropoiesis. The question remains of a polychromosomal lesion of either quiescent F cells or adult stem cells.
X-linked syndrome of platelet dysfunction, thrombocytopenia, and imbalanced globin chain synthesis with hemolysis
Authors: Thompson AR , Wood WG , Stamatoyannopoulos G 
Published: October 2019

Abstract
An unusual family is described with a congenital bleeding disorder present in four males belonging to three generations. Of the three surviving affected males, all had splenomegaly and petechiae. The three had moderate thrombocytopenia (55–90 X 10(9)/liter) and markedly prolonged Ivy-template bleeding times (greater than 30 min). They were also noted to have reticulocytosis and, upon further investigation, imbalanced globin chain synthesis resembling that of beta-thalassemia minor. Studies on nine additional family members in four generations were normal except for slight elevations of reticulocyte counts in female members, one of whom had the abnormal globin chain synthesis ratio. In male members, the bleeding tendency and clinical signs always occurred in the presence of the globin chain synthesis defect and reticulocytosis. This previously undescribed condition was apparently transmitted as an X-linked disorder.