bluuebunny/crossref_metadata_2025_split huggingface dataset:
Carrier detection in hemophilia A: a cooperative international study. II. The efficacy of a universal discriminant
Authors: Green PP , Mannucci PM , Briet E , Ljung R , Kasper CK , Essien EM , Chediak J , Rizza CR , Graham JB 
Published: October 2019

Abstract
Factor VIII (F.VIII) and von Willebrand factor (VWF):Ag data collected by eight laboratories on a total of 336 obligatory carriers of hemophilia A and 137 normal women were used to answer several questions concerning the construction of linear discriminants for carrier detection. It was found: that a “universal” linear discriminant can be constructed which is suitable for use in all laboratories and is nearly as effective as laboratory-specific discriminants; that inclusion of age and ABO blood type data improved the efficacy of these discriminants; that substitution of alternative assays for F.VIII and VWF:Ag did not generally improve the efficacy of the discriminants over that obtained using the bioassay for F.VIII:C and Laurell's immunoassay for VWF:Ag; that linear discriminants were far more effective than discriminants based on the F.VIII:C/VWF:Ag ratio. A step-wise procedure is given which any laboratory may follow in using the universal discriminant for carrier detection.
Cytogenetic features and serum lactic dehydrogenase level predict a poor treatment outcome for children with pre-B-cell leukemia
Authors: Pui CH , Williams DL , Kalwinsky DK , Look AT , Melvin SL , Dodge RK , Rivera G , Murphy SB , Dahl GV 
Published: October 2019

Abstract
Leukemic cells from 89 (24%) of 369 children with newly diagnosed acute lymphoblastic leukemia (ALL) were found to have a pre-B immunophenotype. By comparison with blasts having the common ALL phenotype, the pre-B cells were more likely to have a DNA index less than 1.16 (P = 0.02), a pseudodiploid karyotype (P less than 0.001), and a chromosomal translocation (P = 0.001). Increased serum lactic dehydrogenase levels (P = 0.001) were also characteristic of pre-B ALL; otherwise, the clinical and laboratory features of the two groups were similar. A nonrandom chromosomal translocation, t(1;19)(q23;p13.3), was identified in blast cells from 16 (23%) of the 70 patients with pre-B ALL and adequate chromosome banding studies; different translocations were found in 11 of the remaining patients. The presence of any chromosomal translocation in the pre-B group was significantly related to a higher leukocyte count, an increased level of serum lactic dehydrogenase, an increased percentage of S-phase cells, black race, and a blast cell DNA index less than 1.16. Four presenting features were found to confer an increased risk of treatment failure among pre-B patients: pseudodiploidy, chromosomal translocation, black race, and higher serum lactic dehydrogenase level. In a multivariate analysis, pseudodiploidy emerged as the strongest factor for predicting relapse in pre-B ALL. The frequent association of chromosomal abnormalities of known adverse prognostic significance and high serum lactic dehydrogenase levels with pre-B-cell ALL explains, at least in part, the poor treatment outcome reported for children with this subtype of leukemia.
Induction of Tac antigen and proliferation of myeloid leukemic cells by ATL-derived factor: comparison with other agents that promote differentiation of human myeloid or monocytic leukemic cells
Authors: Yamamoto S , Hattori T , Matsuoka M , Ishii T , Asou N , Okada M , Tagaya Y , Yodoi J , Takatsuki K 
Published: October 2019

Abstract
We studied seven cases of myeloid leukemia at various differentiation stages to investigate the response of leukemic cells to phorbol 12- myristate 13-acetate (PMA) and various biological factors. gamma- Interferon (gamma-IFN)-treated cells expressed higher amounts of Fc receptors on leukemic cells in five out of seven cases. Expression of HLA-DR antigen of gamma-IFN-treated leukemic cells was significantly enhanced in three cases. PMA did not induce Fc receptors or HLA-DR antigen on these cells. Induction of Tac antigen, a putative interleukin 2 (IL 2) receptor, was observed in two cases after cultivation with PMA or with a novel lymphokine, adult T cell leukemia- derived factor (ADF). Cells from one of these patients expressed Tac antigen immediately after cell separation, and expression of Tac antigen was augmented by PMA and ADF. Interleukin 1 (IL 1) or IL 2 did not induce Tac antigen. Leukemic cells from this patient also proliferated vigorously in the presence of ADF but not PMA, IL 1, or IL 2.
Enhancement of the proliferation of human marrow erythroid (BFU-E) progenitor cells by prostaglandin E requires the participation of OKT8- positive T lymphocytes and is associated with the density expression of major histocompatibility complex class II antigens on BFU-E
Authors: Lu L , Pelus LM , Broxmeyer HE , Moore MA , Wachter M , Walker D , Platzer E 
Published: October 2019

Abstract
The relationship between major histocompatibility complex class II antigens (MHC class II, eg, HLA-DR, Ia), T lymphocytes, and the enhancement of erythroid colony formation from BFU-E by prostaglandin E was analyzed using normal bone marrow cells. In primary methylcellulose culture, the addition of prostaglandin E1 (PGE1) to unseparated buffy coat, low-density, or nonadherent low-density (NAL) marrow cells resulted in an enhancement of the total number of erythroid (BFU-E) colonies observed. Treatment of bone marrow cells with a monoclonal antihuman MHC class II antibody plus complement (C') resulted in a reduction of the total number of colonies by approximately 50% and abrogation of the enhancing effect of PGE1. Analysis of accessory cell requirements by depletion of both adherent cells and sheep erythrocyte rosetting lymphocytes (E+ cells) and reconstitution using C' or anti- MHC class II antibody plus C'-treated T cell-depleted NAL (NALT-) marrow cells and E+ cell populations treated with C' or anti-MHC class II antibody plus C' demonstrated a requirement for MHC class II antigen- T cells, but not adherent cells, and a requirement for MHC class II antigen + BFU-E in order to observe the enhancing effect of PGE1 on erythroid colony formation. Positive selection of BFU-E in NALT- bone marrow expressing differing density distributions of MHC class II antigens was accomplished with monoclonal anti-MHC class II antibodies and sorting with a fluorescence-activated cell sorter (FACS). Addition of E+ cells to the different populations of MHC class II antigen+ NALT- cells demonstrated that the PGE-enhancing effects on erythroid colony formation were directly related to increasing density distributions of MHC class II antigens on BFU-E. Colony formation by BFU-E expressing a low density distribution of MHC class II antigens or having no detectable MHC class II antigens, as determined by FACS analysis, was not enhanced by PGE1 in the presence of MHC class II antigen-positive or -negative T cells.
DDAVP infusion in five patients with type Ia glycogen storage disease and associated correction of prolonged bleeding times
Authors: Marti GE , Rick ME , Sidbury J , Gralnick HR 
Published: October 2019

Abstract
Five patients with glycogen storage disease type I (GSD-I) were evaluated for a bleeding diathesis and subsequently were given an infusion of 1-deamino-8-D-arginine vasopressin (DDAVP). Although platelet counts were normal or slightly elevated, the baseline template bleeding times were prolonged in four of the patients. Prothrombin times and activated partial thromboplastin times were normal, while ADP- and epinephrine-induced platelet aggregations were absent in the three patients tested. Ristocetin- and collagen-induced platelet aggregations were abnormal. Laurell and immunoradiometric determinations of the factor VIII-related antigen (vWf antigen) were decreased. Glyoxyl agarose gel electrophoresis of the patients' plasma revealed abnormal multimer patterns in four of the five patients. After the DDAVP infusion the platelet aggregation abnormalities persisted; however, the bleeding time and the von Willebrand antigen and activity corrected. We conclude that GSD-Ia patients may have a metabolically acquired form of von Willebrand's syndrome as well as an acquired intrinsic platelet defect, and that DDAVP may be useful in the management of bleeding in these patients.
Monoclonal antibodies against SU-DHL-1 cells stain the neoplastic cells in true histiocytic lymphoma, malignant histiocytosis, and Hodgkin's disease
Authors: Hsu SM , Pescovitz MD , Hsu PL 
Published: October 2019

Abstract
Three murine monoclonal antibodies, named 2H9, 1E9 and 1A2, were produced after immunization of BALB/c mice with cells of the SU-DHL-1 cell line from a true histiocytic lymphoma. In frozen sections from various lymphomas, 2H9 and 1A2 selectively stained the cell membranes of neoplastic cells in true histiocytic lymphoma and Hodgkin's disease. Antibody 1E9 stained the nuclear membranes of the tumor cells in true histiocytic lymphoma and malignant histiocytosis. No staining was seen in 56 cases of B and T cell lymphoma. Several tissue culture cell lines, including T cell acute lymphoblastic leukemia and pre-B cell lines, were not stained. With 2H9, however, a positive reaction was noted for two Epstein-Barr virus (EBV)-positive African Burkitt's lymphoma cell lines (Daudi and P3HRI), one human T cell lymphoma/leukemia-virus-positive cell line (HUT 102), and one EBV- transformed normal B lymphoblastoid cell line (RPMI 8057). In normal lymphoid tissues, 2H9 and 1E9 reacted with the nuclear membranes of histiocytes and interdigitating reticulum cells, whereas 1A2 stained only rare cells of an unknown type. All three antibodies failed to react with B or T cells in frozen tissue sections of normal lymphoid tissues. The use of these three antibodies should facilitate the diagnosis of histiocyte and interdigitating reticulum (IR) cell-related neoplasms, namely, true histiocytic lymphoma, malignant histiocytosis, and Hodgkin's disease. True histiocytic lymphoma and Hodgkin's disease exhibit similar reactivities with these three and with two other monoclonal antibodies (HeFi-1 and Tac), suggesting that these two types of lymphoma are related. In contrast, malignant histiocytosis was negative for 2H9, 1A2, Tac, and HeFi-1. The difference in the phenotypic expression of true histiocytic lymphoma and malignant histiocytosis indicates that they are two different disease entities.
Functional characterization of platelet-bound factor XIa: retention of factor XIa activity on the platelet surface
Authors: Walsh PN , Sinha D , Koshy A , Seaman FS , Bradford H 
Published: October 2019

Abstract
Previously we have shown that both factor XI and factor XIa are bound specifically to distinct, high-affinity sites on the surface of activated platelets in the presence of high Mr kininogen. To determine the functional significance of factor XIa binding to platelets, bound factor XIa has now been compared with the unbound enzyme. Platelets incubated with thrombin, high Mr kininogen, and 125I-labeled factor XIa bound 130 to 500 molecules of factor XIa per platelet. Scatchard analysis of binding data give a dissociation constant (Kd) of 822 pmol/L +/- 140 (SEM). Rates of factor IX activation, assayed by release of trichloroacetic acid-soluble 3H-labeled activation peptide from purified [3H]-factor IX, were similar when factor XIa was bound to platelets and when it was free in solution. The platelet-bound factor XIa was isolated by centrifugation through 20% sucrose and was functionally characterized both in a factor XIa coagulation assay and in the factor IX activation peptide release assay in comparison with unbound factor XIa in the presence of treated platelets. The functional activity of platelet-bound factor XIa as a factor IX activator as well as its structural integrity were shown to be fully retained on the platelet surface. Since platelets bind factor XI and promote its proteolytic activation to factor XIa, factor XIa binding to platelets may serve to localize factor IX activation to the hemostatic plug, where factor XIa is protected from inactivation by plasma protease inhibitors and where acceleration of subsequent coagulation reactions can occur.
Fragment D-dimer levels: an objective marker of vaso-occlusive crisis and other complications of sickle cell disease
Authors: Devine DV , Kinney TR , Thomas PF , Rosse WF , Greenberg CS 
Published: October 2019

Abstract
Although abnormalities in coagulation tests have been reported during vaso-occlusive crises in patients with sickle cell disease, objective, readily performed laboratory tests that document the occurrence of this complication have not been available. We examined the relationship between fibrin D-dimer levels and the occurrence of complications in patients with sickle cell disease, using a commercially available latex bead agglutination assay. The patients were either asymptomatic, hospitalized for vaso-occlusive crisis, or had other complications of sickle cell disease including leg ulcers, chronic cholecystitis, aseptic necrosis, joint pain and infection. Fifty-seven percent of 187 samples on 96 patients had elevated levels of fibrin D-dimer. Ninety percent of 75 samples from asymptomatic patients were negative for fibrin D-dimer (less than 1 microgram/ml) but 97% of 29 samples from patients with vaso-occlusive crisis and 85% of 83 samples from patients with other complications of sickle cell disease were positive. In serial studies, worsening or amelioration in clinical complications were reflected in increasing or decreasing levels of fibrin D-dimer, respectively. The molecular species of fibrin identified by the latex agglutination test was shown to be fragment D-dimer by successive immunoprecipitation and protein blot analysis. We conclude that the complications of sickle cell disease, including vaso-occlusive crisis, result in the production of fibrin D-dimer, and its detection may be used as a marker for the presence of the complication.
Effects of verapamil on in vitro intracellular accumulation and retention of daunorubicin in blast cells from patients with acute nonlymphocytic leukemia
Authors: Ross DD , Joneckis CC , Schiffer CA 
Published: October 2019

Abstract
The effects of verapamil on the intracellular pharmacokinetics of daunorubicin (DNR) in blast cells from the bone marrows of patients with acute nonlymphocytic leukemia (ANLL) were studied to determine whether verapamil was capable of enhancing intracellular accumulation and retention of DNR in ANLL, as has been observed in murine P388 cells resistant to DNR. Seventeen marrows from ANLL patients were studied, 13 of which were from patients who were considered to be clinically refractory to DNR. We took care to include such patients in this study since, in the P388 model, verapamil enhancement of DNR uptake is observed only in cells resistant to DNR. Intracellular accumulation of DNR was studied by exposing blast cells to DNR (1 microgram/mL) +/- verapamil (6.6 mumol/L) for up to 4 hours. Following a 2-hour exposure of cells to DNR +/- verapamil, intracellular retention of DNR was studied by incubating the cells in DNR-free medium for 3 hours. Intracellular DNR/10(6) cells was quantified by fluorometry. In 12 of 15 patient marrows, verapamil failed to enhance intracellular accumulation of DNR. Three patient marrows had a very small increment in DNR uptake in response to verapamil (approximately 14% greater than DNR alone) that was significant (P less than .05) by paired t test. Intracellular retention of DNR (t 1/2) and the area under the intracellular DNR v time curve (AUC) were studied in 17 patients marrow specimens. No significant alterations in these parameters were observed in response to verapamil. These data indicate either that verapamil did not substantially enhance DNR uptake or retention in blast cells obtained from ANLL patients who are clinically resistant to DNR, or that the frequency of DNR-resistant cells (ie, verapamil-responsive cells) among the blast cells obtained from these patients was too low to influence the population mean of intracellular DNR as measured in these studies.
Increased infection mortality and decreased neutrophil migration due to a component of an artificial blood substitute
Authors: Lane TA , Lamkin GE 
Published: October 2019

Abstract
We previously showed that an artificial blood substitute containing perfluorocarbons, Fluosol-DA, inhibited both neutrophil migration and adherence, due to its detergent component, Pluronic F-68. The purpose of the studies we report here was to determine if Fluosol or Pluronic might also reduce in vivo neutrophil migration and impair host resistance to bacterial infection. We studied in vivo PMN migration by injecting mice intraperitoneally (IP) with glycogen, followed by intravenous (IV) infusion of saline, Fluosol, or Pluronic. Peritoneal lavage after eight hours showed a significant decrease in the accumulation of PMN in lavage fluids of animals given either Fluosol or Pluronic (control--.19 +/- .03 X 10(6) PMN/mL, glycogen--1.35 +/- .14; glycogen/Fluosol--0.63 +/- .12; glycogen/Pluronic--0.69 +/- .07). We ascertained the effect of Fluosol and Pluronic on infection mortality by injecting mice IV with saline, Fluosol, or Pluronic, followed by a quantity of E coli (0.6 X 10(7] IP shown in preliminary studies to kill 20% to 50% of the mice in 24 hours. The 24-hour mortality was 14/45- saline, 24/32-Fluosol (chi 2 = 17.1; P less than .001) and 17/23 - Pluronic (chi = 11.2; P less than .001). Neither Fluosol nor Pluronic caused mortality without E coli. The increase in infection mortality occurred when Fluosol was given either two hours before, or simultaneously with E coli, but only with the simultaneous administration of bacteria and Pluronic. Pluronic did not alter reticuloendothelial system (RES) clearance function. These studies indicate that, in an animal model, Fluosol-DA, due to its detergent component Pluronic F-68, impaired neutrophil delivery to an inflammatory locus, and resulted in an increased rate of infection mortality. Since Pluronic did not result in RES blockade, but did impair the delivery of PMN to an inflammatory locus, our results suggest that the latter effect is responsible for the increase in infection mortality.
Coagulation-cancer interaction in situ in renal cell carcinoma
Authors: Zacharski LR , Memoli VA , Rousseau SM 
Published: October 2019

Abstract
Fibrin was detected by immunospecific techniques associated with both intravascular and extravascular tumor deposits in renal cell carcinoma. In addition, coagulation factors VII and X were demonstrated in intercellular spaces within tumor tissue and adjacent to the surface of tumor cells. Scant accumulation of platelets on intravascular tumor masses was observed. These data suggest that tumor cells in renal cell carcinoma may induce fibrin formation locally by a factor VII-mediated (and thus tissue factor-initiated) pathway of blood coagulation. This mechanism may also account for the hypercoagulable state that exists with this tumor type. We postulate that local fibrin formation may contribute to the growth and spread of this particular type of cancer and that the course of renal cell carcinoma may be ameliorated by anticoagulant therapy.
Acute lymphoblastic leukemia with unusual cytoplasmic granulation: a morphologic, cytochemical, and ultrastructural study
Authors: Fradera J , Velez-Garcia E , White JG 
Published: October 2019

Abstract
The classification of the acute leukemias depends mainly on the morphologic and cytochemical evaluation of the blast forms. One of the main accepted morphologic criteria in the differentiation between acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML) is the absence of granules in the blast cells of ALL. We evaluated a patient with ALL in whom granules were present in the cytoplasm of 35% of the blast cells, as seen in AML. Cytochemical evaluation was performed, including periodic acid-Schiff reaction, Sudan black B, alpha-naphthyl acetate, alpha-naphthyl butyrate, naphthol AS-D chloroacetate, and acid phosphatase stains. The results of these studies confirmed the morphologic impression and diagnosis of ALL. Ultrastructural evaluation revealed that the granules consisted of many tiny vesicles closely packed together in a proteinaceous matrix, resembling to some extent the inclusions described in lymphocytes in the Chediak-Higashi syndrome, but clearly different. The morphologic, cytochemical, and ultrastructural studies of this unique case are presented in detail. To our knowledge, this is the first time that such granules have been described in blast cells of ALL.
The malignant cells in a Lennert's lymphoma are T lymphocytes with a mature helper surface phenotype. A multiparameter flow cytometric analysis
Authors: Stonesifer KJ , Benson NA , Ryden SE , Pawliger DF , Braylan RC 
Published: October 2019

Abstract
The flow cytometric analysis of DNA content in cells obtained from a case of Lennert's lymphoma demonstrated the presence of a discrete hypotetraploid cell population. Correlated multiparameter analysis of DNA, light scatter, and surface antigens by flow cytometry showed that the hypotetraploid cells were intermediate to large cells expressing T11, T3, and T4 antigens and lacking B1 and T8 antigens. These findings suggest that Lennert's lymphoma represents a malignant neoplasm of T- helper lymphocytes.
Treatment of hairy cell leukemia with recombinant alpha-interferon
Authors: Quesada JR , Hersh EM , Manning J , Reuben J , Keating M , Schnipper E , Itri L , Gutterman JU 
Published: October 2019

Abstract
Thirty patients with hairy cell leukemia were treated with recombinant interferon alpha-A (rIFN alpha A; Roferon-A); seven were previously untreated. Nine complete and 17 partial remissions were documented by bone marrow core biopsies. All patients' peripheral blood hematologic indexes either improved or normalized. Twelve of 13 patients with retroperitoneal or mediastinal adenopathy obtained remissions of tumor masses. All seven patients with splenomegaly showed prompt reduction in the size of the spleen to normal size. The incidence of complete remissions was significantly higher (P = .02) in previously untreated patients (5 of 7) than in those in whom splenectomy had been performed (4 of 23), a result presumed to be related to the pretreatment tumor burden. rIFN alpha A was well tolerated; mild fatigue was the most frequent complaint. In most patients, tumor remissions resulted in an improved quality of life: they eliminated the need for transfusing blood products and reduced the incidence of infections, and immune deficits were apparently restored in some of the patients. We conclude that rIFN alpha A is an effective therapy for all stages of hairy cell leukemia including previously untreated or newly diagnosed patients.