bluuebunny/crossref_metadata_2025_split huggingface dataset:
Comparison of laboratory tests used for identification of the lupus anticoagulant
Authors: Lo Samuel C. L. , Oldmeadow Michael J. , Howard Margaret A. , Firkin Barry G. 
Published: February 2007

AbstractA comparison of the sensitivities of the ten most commonly used tests for the identification of the lupus anticoagulant (LA) and the lupus cofactor phenomenon was undertaken on 18 patients. All investigations, except the cardiolipin‐antibody ELISA assay, were carried out using patient's plasma alone followed by a 1:1 mix with control plasma. Dilution studies (1:3, 1:6, 1:9—patient:control) were also carried out. The kaolin clotting time (KCT) was the only test positive in all patients at all dilutions, while the dilute activated partial thromboplastin time with kaolin (Dil‐APTT) registered 17 of 18 positive at all dilutions. Both the dilute Russell viper venom time (Dil‐RVVT) and the tissue thromboplastin inhibition time (TTI) (1/500 thromboplastin) identified the LA in 17 of 18 patients on initial testing but were less sensitive in the dilution studies. The KCT is not a suitable test for routine laboratory use, as it requires an individual filtration step. Therefore a combination of either the Dil‐APTT or Dil‐RVVT together with the TTI (1/500 dilution thromboplastin) is recommended for routine LA screening, as all patients with LA in this study were identified using these easily automated tests. The lupus cofactor phenomenon was most frequently demonstrated using the Dil‐APTT.
8;21 translocation in myelodysplasia secondary to essential thrombocythemia
Authors: Knottenbelt Elaine , Hallett Janet , Jacobs Peter 
Published: February 2007

AbstractA case is presented of a 73‐year‐old woman who received busulphan for essential thrombocythemia and subsequently developed a myelodysplastic syndrome (MDS), which transformed to acute nonlymphoblastic leukaemia within 1 month. Cytogenetic studies showed a 46,XX,t(8;21) (q22;q22) karyotype in all metaphases examined at diagnosis. The karyotypic abnormality is previously unreported in secondary myelodysplasia and may have specific clinical implications in this setting, such as early transformation to acute leukaemia and short survival. This finding contrasts with the generally favourable prognosis of the 8;21 translocation in patients with de novo acute nonlymphoblastic leukemia. A possible explanation for this difference may be the involvement of a committed progenitor in acute nonlymphoblastic leukaemia, while in myelodysplasia the more primitive multipotent stem cell may be affected.
Thrombocytopenia associated with auranofin therapy: Evidence for a gold‐dependent immunologic mechanism
Authors: Kosty Michael P. , Hench P. Kahler , Tani Patricia , McMillan Robert 
Published: February 2007

AbstractThrombocytopenia associated with gold therapy is thought to be due to an immunemediated mechanism. Relatively few patients have been studied so far, and precise details of the pathophysiology of this disease remain undetermined. We report a patient with gold‐induced thrombocytopenia resulting from auranofin therapy. The patient's plasma contained platelet‐reactive antibodies detectable only in the presence of gold salts. Antibody binding occurred at gold concentrations ranging from 0.01 to 10,000 ng/ml. The binding occurred independently of gold salt used, suggesting that substitution of a different gold preparation in this patient would result in a similar thrombocytopenia. These data support a drug‐dependent immune mechanism for platelet destruction.
Vasculitis in hairy cell leukemia: Rapid response to interferon alpha
Authors: Gómez‐Almaguer David , Herrera‐Garza José Luis , Garcia‐Guajardo Blanca M. , Garcia‐Taboada Blanca E. 
Published: February 2007

AbstractA patient with hairy cell leukemia (HCL) and vasculitis was treated only with interferon alpha‐2b. A good, rapid response was obtained without using corticosteroids or immunosuppressors. Interferon may prove to be useful for the systemic therapy of patients with vasculitis associated with HCL.
Different sensitivities of rat and human red cells to exogenous Ca2+
Authors: Swislocki Norbert I. , Tierney Joan M. 
Published: February 2007

AbstractDuring an examination of the effects of shear and of the Ca2+ ionophore A23187 on Ca2+ entry into erythrocytes of rats and humans, we noted that rat erythrocytes were much more sensitive to Ca2+‐induced hemolysis than the human cells. An examination of the effect of Ca2+ on transglutaminase, a cytosolic enzyme in the erythrocyte which crosslinks membrane proteins and renders cells less deformable, demonstrated a correlation between enzyme activity and Ca2+‐induced hemolysis. Both rat and human cells subjected to shear‐induced Ca2+ entry exhibited increased enzyme activity and altered membrane protein SDS‐PAGE patterns. Twenty micromolar A23187 with Ca2+ at concentrations above 80 μM caused hemolysis of rat erythrocytes. In contrast to human erythrocytes, under these conditions no membranes were recoverable from rat erythrocytes. At lower concentrations of Ca2+ (25 and 50 μM), however, rat erythrocytes maintained integrity, and exhibited enhanced transglutaminase activity and cross‐linking of membrane proteins. The rat enzyme can be activated 30% by 10 μM Ca2+, while 50 μM Ca2+ was necessary to achieve a similar activation of the enzyme from human red blood cells. In studies of shear‐stimulated Ca2+ uptake by erythrocytes the rat red cell enzyme was more readily activated. The SDS‐PAGE pattern of rat red cell membranes after a 30 sec shear showed specific changes in protein banding, including the appearance of bands >330 kDa. Changes in protein banding were also apparent in cytosolic proteins. This work supports the view that shear‐induced Ca2+ entry activates transglutaminase that leads to cross‐linking of membrane components, a loss of cell integrity, and eventual cell death.
Iron metabolism in normal and hemochromatotic macrophages
Authors: Baynes Roy D. , Bukofzer Gail , Bothwell Thomas H. , Meyer Theo E. , Friedman Brian M. , Macfarlane Bruce J. , Lamparelli Rosario D. 
Published: February 2007

AbstractCertain metabolic pathways of iron were studied in macrophages (cultured human monocytes) obtained from normal and hemochromatotic subjects. The relative abilities of the hydrophobic ferrous chelator 2,2′ bipyridine and the hydrophilic ferric chelators desferrioxamine (DFO) and diethylenetriaminepenta‐acetic acid (DTPA) to release iron from normal and hemochromatotic macrophages which had previously been loaded with diferric transferrin were tested but there were no differences between the two groups. The relative affinity of the macrophages for diferric transferrin was next studied. Although the hemochromatic macrophages had a somewhat lower affinity for diferric transferrin iron than normal macrophages (Kd 4.7 × 10−8 M vs. 3.0 × 10−8 M) the difference did not reach statistical significance (t = 2.01013; P < 0.07). In a further experiment there was no evidence that apotransferrin was directly involved in the release of iron from hemochromatotic macrophages. A clue to the nature of postendocytotic trans‐membrane transport of iron was provided by the finding that it was inhibited by the hydrophobic ferrous chelator 2,2′ bipyridine. However, the degree of inhibition was similar in both normal and hemochromatotic macrophages. In summary, none of the metabolic processes examined in the present study was abnormal in cultured human blood monocytes from hemochromatotic subjects.
Subcutaneous desmopressin (DDAVP) shortens the bleeding time in uremia
Authors: Vigano Gian Luigi , Mannucci Pier Mannuccio , Lattuada Antonella , Harris Alan , Remuzzi Giuseppe 
Published: February 2007

AbstractThe intravenous infusion of 1‐deamino‐8‐D‐arginine vasopressin (DDAVP) is used as a nontransfusional form of treatment in patients with congenital and acquired bleeding disorders, including patients with uremia associated with prolonged bleeding times. Since uremic patients experience minor bleeding episodes that might be self‐managed at home (particularly epistaxis, gingival bleeding, and menorrhagia), we carried out a double‐blind, placebo‐controlled crossover study in nine uremics to evaluate whether the prolonged bleeding times could be shortened by subcutaneous injections of DDAVP. One hour after administration, the bleeding time was significantly shortened (P < .01) and became normal in seven of nine patients. After 4 hr, the bleeding time was still shorter than baseline (P < .01), but in only three patients was it still normal. There was no significant bleeding time change after placebo. When the same patients were treated with the same dose of DDAVP infused intravenously, the bleeding times were not significantly different from those measured after subcutaneous administration. Hence, subcutaneous DDAVP is an alternative method for short‐term shortening of the bleeding time in uremia, at least as effective as intravenous DDAVP but with the possibility of self‐administration by the patients at home.
Corticosteroid‐responsive pure red cell aplasia in rheumatoid arthritis and its association with pregnancy: A case report
Authors: Banavali S. D. , Parikh P. M. , Charak B. S. , Agarwala S. , Modi A. , Iyer R. , Saikia T. , Gopal R. , Advani S. H. 
Published: February 2007

AbstractPure red cell aplasia (PRCA) often occurs secondary to drug therapy for rheumatoid arthritis (RA). However, idiopathic PRCA in RA is very rare. Though different immunosuppressive therapies have been tried in the past with variable responses, there has been no case report in adults of favourable response to corticosteroids alone. We report a rare case of PRCA in RA, which responded to steroid therapy. Subsequently, the patient relapsed twice, during the first trimester of consecutive pregnancies. The association of PRCA with RA and pregnancy is discussed.
Suppression by bone marrow but not by blood T lymphocytes of autologous and allogeneic granulocytic stem cells in a case of lymphoproliferative T‐cell syndrome
Authors: Marinof Christine , Clément Serge 
Published: February 2007

AbstractThe bone marrow T lymphocytes of a patient suffering from a lymphoproliferative T‐cell syndrome suppressed the in vitro growth of autologous and allogeneic CFU‐GM. The patient's blood T lymphocytes had no suppressor effect on these same stem cells.
Granulocyte transfusion therapy and amphotericin B: Adverse reactions?
Authors: Dutcher Janice P. , Kendall Joyce , Norris Dottie , Schiffer Charles , Aisner Joseph , Wiernik Peter H. 
Published: February 2007

AbstractOne hundred twenty‐five granulocyte transfusions were given concurrently with amphotericin B to 31 granulocytopenic patients with acute leukemia during a four year period. Twenty‐six patients had culture‐documented, and 5 had presumed fungal infections; pulmonary infiltrates were present in 26 patient courses. Eight patients developed pulmonary deterioration temporally related to therapy with amphotericin, granulocyte transfusions, or both. One event occurred following amphotericin alone. Three additional reactions occurred in alloimmunized patients with antibodies to human leukocyte antigens (HLA) who received random donor granulocytes, which may indicate a potential mechanism for the pulmonary reactions. Two reactions potentially represent an adverse interaction between amphotericin and granulocytes, but these were reversible and were not unlike reactions expected with each modality alone. Our data fail to document a specific detrimental interaction between granulocyte transfusions and amphotericin beyond the reactions associated with each modality, and the data suggest that other clinical factors, particularly infection and alloimmunization, also contribute to pulmonary decompensation. We nevertheless recommend great care and attention be given to administering these modalities in the setting of severely ill patients.
Modified osmotic fragility test for the laboratory diagnosis of hereditary spherocytosis
Authors: Judkiewicz Luba , Bartosz Grzegorz , Oplatowska Anna , Szczepanek Andrzej 
Published: February 2007

AbstractA modified osmotic fragility test, based on measurement of hemolysis in four hypotonic NaCl solutions and logarithmic linearization of osmotic fragility curve is, like the “Pink test,” a specific and sensitive test for the laboratory diagnosis of hereditary spherocytosis.
Further studies on the use of serum gastrin levels in assessing the significance of low serum B12 levels
Authors: Miller A. , Slingerland D. W. , Cardarelli J. , Burrows B. A. 
Published: February 2007

AbstractThe reported incidence of low serum vitamin B12 levels in patients ranges from 4% to 8%. In only a small percentage can this be ascribed to readily diagnosed pernicious anemia, malabsorption syndrome, or to gastrointestinal surgery. This leaves a number of patients in whom the meaning of the low serum B12 is not immediately apparent. In 71 patients with intact gastrointestinal tracts, hematological indices did not adequately separate patients with decreased absorption of unbound B12 from those with normal absorption. A low absorption of B12, either unbound or food‐bound, was found, however, when the level of serum gastrin was elevated. In contrast, a normal absorption of unbound B12 was likely (95% of 44 patients) when the serum gastrin was normal.
Successful treatment of severe refractory aplastic anemia with 3‐β etiocholanolone and nandrolone decanoate
Authors: Seewald T. R. , Zeigler Z. R. , Gardner F. H. 
Published: February 2007

AbstractA patient with aplastic anemia, who had been unresponsive to androgens, antithymocyte globulin, high‐dose methylprednisolone, and cyclosporine, responded to treatment with 3‐β‐etiocholanolone, nandrolone decanoate, and prednisolone acetate. Six months following intiation of therapy, she became red cell and platelet transfusion independent with neutrophils persistently over 1,000/μl. A sustained partial remission has persisted for over 2 years. This observation suggests that a combined hematostimulatory approach may be of benefit, even in patients with long‐standing refractory aplasia.
Erythrocyte pyruvate kinase deficiency: A kinetic method for differentiation between heterozygosity and compound‐heterozygosity
Authors: Lakomek Max , Winkler Heinz , Linne Sabine , Schröter Werner 
Published: February 2007

AbstractThe goal of the present study was to search for criteria that allow one to distinguish between normal individuals and heterozygotes as well as compound heterozygotes for pyruvate kinase (PK) deficiency. As the residual activity of PK with heterozygotes was between 35% and 110% of the normal activity, it was necessary to find other methods to prove heterozygosity. The PK in the hemolysates of 23 patients suffering from PK deficiency, 36 paternal and maternal enzymes as well as the enzymes of five heterozygous and four normal siblings together with those of 20 normal individuals, were studied according to the recommendations of the International Committee for Standardization in Haematology. The following hematological and enzyme kinetic parameters can serve to identify heterozygotes for PK deficiency: 1) a slight reticulocytosis, 2) an up‐to‐twofold increase of the intracellular concentrations of glucose‐6‐phosphate in the erythrocyte, 3) a mixed cooperativity of the phosphoenolpyruvate (PEP)‐binding process of PK, 4) a decreased nucleotide specificity with guanosine diphosphate and uridine diphosphate, and 5) a lowered affinity for adenosine diphosphate. The most significant criterium found with all heterozygotes was a mixed cooperativity of the PEP‐binding process caused by the presence of a mixture of normal and mutant PK.
Heterogeneity in the breakpoints of chromosome 19 among acute leukemia patients with the t(11;19)(q23;p13) translocation
Authors: Mitani Kinuko , Sato Yuko , Kobayashi Yukio , Shibasaki Yoshikazu , Kasuga Masato , Inaba Toshiya , Hayashi Yasuhide , Miura Yasusada , Miyazono Kohei , Hirai Hisamaru , Urabe Akio , Takaku Fumimaro 
Published: February 2007

AbstractGene probes for insulin receptor (INSR) and c‐ets‐1 were hybridized to metaphase cells from three leukemic patients with the t(11;19)(q23;p13) translocation. Patients 1 and 2 were diagnosed as acute lymphocytic leukemia (ALL) (L2), and patient 3, as acute myelogenous leukemia (AML) (M4). The c‐ets‐1 gene was demonstrated to have translocated from chromosome 11 to the short arm of the rearranged chromosome 19 (19p+) in all three patients. On the other hand, the INSR gene translocated from chromosome 19 to the rearranged chromosome 11 (11q‐) in the AML case, but remained on the rearranged chromosome 19 in the two ALL cases. Thus, the breakpoints of chromosome 19 are different among the patients studied, proximal to the INSR gene locus in the AML case and distal in the two ALL cases. Consequently, the c‐ets‐1 gene and the INSR gene remain separated in the AML case, whereas they become close to each other in the two ALL cases. Rearrangement of these two genes was studied in the two ALL patients, with no positive data being obtained. The results suggest that there may be heterogeneity in the breakpoints of chromosome 19 among the t(11;19)‐associated acute leukemias.
Urokinase therapy for a central venous catheter thrombus
Authors: Suarez Carlos R. , Ow E. Phillip , Lambert George H. , Anderson Craig L. , Purewal Navtej S. 
Published: February 2007

AbstractA 670 g premature infant is described in whom an intracardiac thrombus was documented. This thrombus formation probably resulted as a complication of an indwelling right atrial catheter. Thrombolytic therapy with urokinase was instituted, resulting in total and rapid dissolution. No hemorrhagic complications resulted. We believe that this particular thrombolytic therapy is safe and effective and should be considered when facing this particular complication.
The use of continuous infusion of factor concentrates in the treatment of hemophilia
Authors: Bona Robert D. , Weinstein Ralph A. , Weisman Steven J. , Bartolomeo Ann , Rickles Frederick R. 
Published: February 2007

AbstractBolus Infusion of clotting factor concentrates remains the most common approach to the treatment or prevention of bleeding in patients with hemophilia. Although successful use of continuous infusion of such concentrates has been reported by several groups, this alternative treatment method has not achieved widespread popularity. We report here our experience in one hemophilia center with the use of continuous infusion of factor VIII and factor IX concentrates in 13 patients, 11 with hemophilia A, and 2 with hemophilia B. All patients were treated successfully for bleeding episodes (e.g., hemarthroses, intracranial, or gastrointestinal bleeding) or for surgical procedures (appendectomy, thoracotomy, etc.). Three patients with low titer factor VIII inhibitors were treated successfully with constant infusion therapy, requiring a mean dose of factor VIII concentrate 2.3 fold (8.20 u/kg/h) higher than that of the patients without inhibitors (3.63 u/kg/h) to maintain a circulating plasma level of factor VIII of 1 u/ml.The use of constant infusion of clotting factor concentrates is safe, efficacious, and more convenient than bolus therapy of factor concentrates and should be considered for hospitalized hemophilia patients requiring replacement therapy.
DNA rearrangement and restriction fragment length polymorphism within the first BCR intron in philadelphia‐positive acute leukemia
Authors: Nakamura Yuichi , Miura Osamu , Sugahara Yuichi , Maseki Nobuo , Kaneko Yasuhiko , Aoki Nobuo 
Published: February 2007

AbstractIn some patients with Philadelphia (Ph)‐chromosome positive acute lymphoblastic leukemias, breakpoints on chromosome 22 are reported to occur within the first BCR intron. To analyze the breakpoints in chromosome 22 of Ph‐positive acute leukemia patients without rearrangement of the 5.8 kb bcr, we cloned the 3′ part of the first BCR intron using a synthetic DNA probe. During the course of study, we mapped the region of the deletion/insertion of 1 kb that causes a restriction fragment length polymorphism (RFLP) and found a racial difference in the frequencies of the alleles giving rise to this RFLP. Analyses of the patients' samples indicated that breakpoints were located within the 8.5 kb EcoRI fragment of the first BCR intron in two of five Ph‐positive acute leukemia patients. The data, together with the previous reports, indicate that breakpoints within this ∼50 kb intron are widely scattered, in contrast to those confined within the 5.8 kb bcr in chronic myelogenous leukemias.
Thrombin and plasmin generation in patients with liver disease
Authors: Takahashi Hoyu , Tatewaki Wataru , Wada Ken , Yoshikawa Akira 
Published: February 2007

AbstractPatients with liver disease frequently have multiple hemostatic abnormalities. Coagulation and fibrinolytic factors and inhibitors may decrease as the result of impaired synthesis and/or enhanced catabolism. In order to assess the actual degree of activation of coagulation and fibrinolytic systems in liver disease, plasma levels of thrombin‐antithrombin III complex (TAT) and plasmin‐α2‐antiplasmin complex (PAP) were measured together with cross‐linked fibrin derivatives (XDP), tissue‐type plasminogen activator (t‐PA), and plasminogen activator inhibitor (PAI‐1) in 31 patients with liver disease (five patients with acute hepatitis, seven with chronic hepatitis, nine with liver cirrhosis, and ten with hepatocellular carcinoma). Mean plasma levels of TAT (mean 4.2 ± SD 4.0 μg/L), PAP (0.7 ± 0.7 mg/L), and XDP (374 ± 518 μg/L) were significantly elevated in patients with liver disease as compared with normal subjects (TAT of 1.7 ± 0.3 μg/L, PAP of 0.2 ± 0.1 mg/L, and XDP of 30 ± 14 μg/L; P < 0.005). Plasma concentrations of t‐PA and PAI‐1 antigens were also elevated. When plotted by the disease categories, the magnitude of elevations of these parameters was variable among subgroups. Patients with acute hepatitis had considerably higher TAT levels. The mean PAP values were relatively high in chronic hepatitis and hepatocellular carcinoma, in which an elevation of the t‐PA/PAI‐1 ratio was observed. Although clearance of TAT and PAP should be evaluated in the future, these findings suggest that excessive amounts of thrombin and plasmin are actually generated in patients with liver disease.
Polymerase chain reaction amplification applied to the determination of β‐like globin gene cluster haplotypes
Authors: Sutton Millicent , Bouhassira Eric E. , Nagel Ronald L. 
Published: February 2007

AbstractWe report here on the application of the polymerase chain reaction (PCR) technique for the determination of the β‐like gene cluster haplotypes. Seven fragments containing each one of the following polymorphic sites—Xmnl 5′ to the Gγ gene, Hindlll in the IVSII of Gγ and A γ gene, Hincll 3′ and inside the γ gene, Hinfl 5′ of the β gene—and Hpal 3′ of the β gene—are amplified using the PCR technique. Each amplified fragment is subsequently digested with the appropriate enzyme, analyzed by electrophoresis on agarose gel containing ethidium bromide, and visualized under ultraviolet light. This technique has the advantages of rapidity, safety, and cost‐effectiveness.