Neutrophil elastase in patients with homozygous beta-thalassemia and pseudoxanthoma elasticum-like syndrome.

abstract：:A 37-year-old untransfused, non-drinking man with Hemoglobin H-CS disease presented with insulin-dependent diabetes mellitus, markedly elevated serum ferritin level, and marked iron deposition in hepatocytes. He did not carry either of the two common mutations of the HLA-H gene for hereditary hemochromatosis, namely, ...

journal_title：American journal of hematology

authors： Chim CS,Chan V,Todd D

更新日期：1998-02-01 00:00:00

abstract：:Neutrophils that bear receptors for the Fc portion of immunoglobulin G have been demonstrated to be more active in assays of adherence, aggregation, and chemotaxis compared to Fc receptor-negative cells. We examined the relationship of neutrophil Fc receptor activity and cell-cell adherence or aggregation induced by p...

journal_title：American journal of hematology

authors： Spagnuolo PJ,Fain M,Bass SN

更新日期：1987-11-01 00:00:00

abstract：:Neonatal alloimmune thrombocytopenia is an uncommon but important cause of thrombocytopenia in infants. Because of the severity of the thrombocytopenia, some of these infants will have intracranial hemorrhage with resultant long-term disability. Obstetricians and neonatologists have recommended delivery by caesarean s...

journal_title：American journal of hematology

authors： Burrows RF,Caco CC,Kelton JG

更新日期：1988-06-01 00:00:00

abstract：:Initially described in 1948 by Hertert thromboelastography (TEG) provides a real-time assessment of viscoelastic clot strength in whole blood. Rotational thromboelastometry (ROTEM) evolved from TEG technology and both devices generate output by transducing changes in the viscoelastic strength of a small sample of clot...

journal_title：American journal of hematology

authors： Whiting D,DiNardo JA

更新日期：2014-02-01 00:00:00

abstract：:Eosinophilia may represent an early paraclinical sign of hematological malignant disease, but no reports exist on its predictive value for hematological malignancies. From the Copenhagen Primary Care Differential Count (CopDiff) Database, we identified 356,196 individuals with at least one differential cell count (DIF...

journal_title：American journal of hematology

authors： Andersen CL,Siersma VD,Hasselbalch HC,Lindegaard H,Vestergaard H,Felding P,de Fine Olivarius N,Bjerrum OW

更新日期：2013-10-01 00:00:00

abstract：:Nongastric marginal zone B-cell lymphoma (NG-MZL) is a relatively uncommon indolent lymphoma. From 1990 to 2005, a total of 247 patients with histologically confirmed NG-MZL were analyzed. Ann Arbor stage I/II disease was present in 78% (167 out of 215). One hundred eighty-six patients out of two hundred eight were ca...

journal_title：American journal of hematology

authors： Oh SY,Ryoo BY,Kim WS,Park YH,Kim K,Kim HJ,Kwon JM,Lee J,Ko YH,Ahn YC,Oh SJ,Lee SI,Kim HJ,Kwon HC,Bang SM,Kim JH,Park J,Lee SS,Kim HY,Park K

更新日期：2007-06-01 00:00:00

abstract：:Argentine hemorrhagic fever (AHF) is a viral disease caused by Junin virus and characterized by hematologic and neurological involvement. The main hematologic features are leukopenia, thrombocytopenia, and bone marrow hypoplasia. Hematopoietic growth factors serum levels were measured by ELISA technique in forty-eight...

journal_title：American journal of hematology

authors： Marta RF,Enria D,Molinas FC

更新日期：2000-05-01 00:00:00

abstract：:Cobalamin (vitamin B12) deficiency is a common cause of megaloblastic anemia in Western populations. Laboratory evaluation of megaloblastic anemia frequently includes the assessment of patient cobalamin and folate status. Current total serum cobalamin measurements are performed in the clinical laboratory with competit...

journal_title：American journal of hematology

authors： Oberley MJ,Yang DT

更新日期：2013-06-01 00:00:00

abstract：:The trial CICL670AUS04 was a single-arm, open-label study of the cardiac efficacy of 18 months of deferasirox monotherapy [1]. Cardiac response in this study was related to the degree of liver siderosis. Patients with mild to moderate liver siderosis improved their cardiac T2* while more severely siderotic patients di...

journal_title：American journal of hematology

authors： Wood JC,Glynos T,Thompson A,Giardina P,Harmatz P,Kang BP,Paley C,Coates TD

更新日期：2010-10-01 00:00:00

abstract：:Anemia is a major cause of morbidity and mortality among patients with sickle cell anemia. In this study, 108 episodes of severe anemia were prospectively evaluated in 108 patients with hemoglobin SS disease attending the pediatric sickle cell clinic of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Youn...

journal_title：American journal of hematology

authors： Juwah AI,Nlemadim A,Kaine W

更新日期：2003-03-01 00:00:00

abstract：:This review focuses on the initial clinical descriptions and subsequent investigation of the syndrome of recurrent infections associated with neutrophil membrane glycoprotein deficiencies. Characterization of the missing group of three glycoprotein heterodimers and their role in adhesion-related neutrophil function is...

journal_title：American journal of hematology

authors： Styrt B

更新日期：1989-08-01 00:00:00

abstract：:In this study, we further established the role of interleukin-1 alpha (IL-1 alpha), interleukin-1 beta, tumor necrosis factor-alpha (TNF-alpha), and interferon-alpha (IFN-alpha) as regulators of proliferation of acute myeloid leukemia (AML) cells. AML cells from 8 of 15 patients incorporated high levels of 3H-thymidin...

journal_title：American journal of hematology

authors： Carter A,Silvian-Draxler I,Tatarsky I

更新日期：1992-08-01 00:00:00

abstract：:Six patients and their parents from five different families with Hb H have been evaluated clinically and hematologically. Previous studies using restriction endonuclease mapping technique indicated that alpha-thalassemia determinants in these cases are heterogeneous. Only one of the five cases have the usual genotype ...

journal_title：American journal of hematology

authors： Altay C,Gurgey A,Tuncbilek E

更新日期：1980-01-01 00:00:00

abstract：:Responsiveness of bone marrow erythropoietic stem cells (CFU-E and BFU-E) to recombinant human erythropoietin (rh-Ep) was examined in vitro in 23 patients with aplastic anemia and 14 with myelodysplastic syndrome (MDS) to investigate the clinical use of rh-Ep for these diseases. Bone marrow mononuclear cells were cult...

journal_title：American journal of hematology

authors： Aoki I,Higashi K,Homori M,Chikazawa H,Ishikawa K

更新日期：1990-09-01 00:00:00

abstract：:Little is known about the mechanisms explaining the wide variation in platelet counts (PLT) and other hematologic parameters in humans. We previously showed that the sex-based difference in hematocrit was associated with nucleotide variation in the erythropoietin receptor gene (EPOR). We sought to identify new polymor...

journal_title：American journal of hematology

authors： Zeng SM,Murray JC,Widness JA,Strauss RG,Yankowitz J

更新日期：2004-09-01 00:00:00

abstract：:The mechanism of multinucleated cell formation in Hodgkin's disease has not yet been elucidated. We asked whether the giant multinucleated cells of the H-RS cell line L1236 develop via fusion of the predominant smaller cells. As a positive control for the fusion assay, human B cells from the B-cell lymphoma cell line ...

journal_title：American journal of hematology

authors： Re D,Benenson E,Beyer M,Gresch O,Draube A,Diehl V,Wolf J

更新日期：2001-05-01 00:00:00

abstract：:Because of a possible hazard from the use of radioisotopes to determine iron absorption by infants, the use of stable isotopes for this purpose has much appeal. We have applied the method of inductively coupled plasma mass spectrometry (ICP/MS) to determine the mass ratio, 58Fe/57Fe, in blood before and after oral adm...

journal_title：American journal of hematology

authors： Janghorbani M,Ting BT,Fomon SJ

更新日期：1986-03-01 00:00:00

abstract：:A 50-year-old woman with muscular fatigue and monoclonal gammopathy of unknown significance was shown to have high levels of antibodies against the acetylcholine receptor of the skeletal muscle endplate. The specific antibody activity was exclusively associated with the monoclonal component. There were no objective si...

journal_title：American journal of hematology

authors： Ahlberg RE,Lefvert AK

更新日期：1988-09-01 00:00:00

abstract：:A minority of super-utilizing adults with sickle cell disease (SCD) account for a disproportionate number of emergency department (ED) and hospital admissions. We performed a retrospective cohort study comparing the rate of admission before and after the opening of a clinic for adults with SCD. Unique to this clinic w...

journal_title：American journal of hematology

authors： Koch KL,Karafin MS,Simpson P,Field JJ

更新日期：2015-03-01 00:00:00

abstract：:Hb Saint Nazaire [beta 103 (G5) Phe-->Ile] was found in four apparently unrelated French families. The five patients carrying this hemoglobin have been detected because of a moderate erythrocytosis. The structural abnormality of Hb Saint Nazaire concerns the same residue as in Hb Heathrow [beta 103 (G5) Phe-->Leu). A ...

journal_title：American journal of hematology

authors： Wajcman H,Kister J,M'Rad A,Promé D,Milpied N,Rapp MJ,Harousseau JL,Riou J,Bardakdjian J,Galacteros F

更新日期：1993-09-01 00:00:00

abstract：:The role of peripheral platelet destruction as a reversible etiology of thrombocytopenia in chronic lymphocytic leukemia (CLL) was evaluated in nine patients with CLL and refractory thrombocytopenia who underwent splenectomy. The patients' ages ranged from 54 to 74 years. Progressive thrombocytopenia refractory to ant...

journal_title：American journal of hematology

authors： Merl SA,Theodorakis ME,Goldberg J,Gottlieb AJ

更新日期：1983-11-01 00:00:00

abstract：:Although tyrosine kinase inhibitors have redefined the care of chronic myeloid leukemia (CML), these agents have not proved curative, likely due to resistance of the leukemia stem cells (LSC). While a number of potential therapeutic targets have emerged in CML, their expression in the LSC remains largely unknown. We t...

journal_title：American journal of hematology

authors： Gerber JM,Qin L,Kowalski J,Smith BD,Griffin CA,Vala MS,Collector MI,Perkins B,Zahurak M,Matsui W,Gocke CD,Sharkis SJ,Levitsky HI,Jones RJ

更新日期：2011-01-01 00:00:00

abstract：:Tissue factor pathway inhibitor (TFPI) is a Kunitz-type proteinase inhibitor that has a crucial role in haemostasis and is primarily synthesized in the vascular endothelium. We investigated plasma total TFPI, antiphospholipid antibodies, and some other coagulation and fibrinolytic system parameters in 30 patients with...

journal_title：American journal of hematology

authors： Akarsu M,Demirkan F,Ozsan GH,Onen F,Yüksel F,Ozkan S,Undar B

更新日期：2001-12-01 00:00:00

abstract：:Circulating thrombomodulin is a novel endothelial cell marker, which may reflect the endothelial injury. Plasma levels of thrombomodulin were quantitated by an enzyme-linked immunosorbent assay (ELISA) in patients with hematological malignancies, liver disease, diabetes mellitus, collagen disease, thrombotic disease, ...

journal_title：American journal of hematology

authors： Takahashi H,Ito S,Hanano M,Wada K,Niwano H,Seki Y,Shibata A

更新日期：1992-09-01 00:00:00

abstract：:A rare beta-thalassemia mutation at the splicing junction [namely, G-->C in intervening sequence (IVS) I-1] was found in a Japanese family. The proband and his mother were heterozygous for the mutation. Analysis of mRNA extracted from the reticulocyte-rich fraction obtained from the proband's mother revealed that the ...

journal_title：American journal of hematology

authors： Fujihara N,Tozuka M,Ueno I,Yamauchi K,Nakagoshi R,Ishikawa S,Hirota M,Okumura N,Ishii E,Katsuyama T

更新日期：2003-01-01 00:00:00

abstract：:Quantitative changes of hemostatic molecular markers were studied in patients with nephrotic syndrome. The plasma levels of fibrinopeptide A (FPA), thrombin-antithrombin III complex (TAT), products of thrombin activation, and fragment F1 + 2 (F1 + 2), a product of prothrombin activation, were measured by enzyme immuno...

journal_title：American journal of hematology

authors： Chen TY,Huang CC,Tsao CJ

更新日期：1993-12-01 00:00:00

abstract：:Thrombocytopenia is a poor prognostic indicator in the myelodysplastic syndromes (MDS). Treatment options for patients with symptomatic thrombocytopenia are limited. Danazol, an attenuated androgen, may have some efficacy in increasing the platelet count of patients with MDS. We retrospectively reviewed 33 patients wi...

journal_title：American journal of hematology

authors： Chan G,DiVenuti G,Miller K

更新日期：2002-11-01 00:00:00

abstract：:In addition to transferrin, ferritin can also function as a source of iron for heme synthesis (Gelvin D, et al. Blood 1996;88:3200-3207; Meyron-Holtz EG, et al. Blood 1999;94:3205-3211). In the present study we investigate the effect of external ferritin on the proliferation and hemoglobinization of human erythroid pr...

journal_title：American journal of hematology

authors： Leimberg JM,Konijn AM,Fibach E

更新日期：2003-07-01 00:00:00

abstract：:The 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 ...

journal_title：American journal of hematology

authors： Lakomek M,Winkler H,Linne S,Schröter W

更新日期：1989-08-01 00:00:00

abstract：:High-resolution two-dimensional gel electrophoresis (2-DGE) was used to analyse plasma samples and partially purified cold agglutinins (CA) obtained from two selected patients. Both presented an acute hemolytic anemia with CA of high thermal amplitude, normal immunoglobulin levels, no detectable paraproteinemia, and n...

journal_title：American journal of hematology

authors： Tissot JD,Clément F,Schifferli JA,Frei PC,Hochstrasser DF,Schneider P

更新日期：1992-07-01 00:00:00