bluuebunny/crossref_metadata_2025_split huggingface dataset:
Allogeneic bone marrow transplantation for hematological malignancies following etoposide, cyclophosphamide, and fractionated total body irradiation
Authors: Yau Jonathan C. , Huan Susan D. , Lemaistre Charles F. , Meneghetti Carole M. , Andersson Borje S. , Wallerstein Ralph O. , Woo Shiao Y. , Brunner Lane J. , Dimopoulos Meletios A. , Jagannath Sundar , Deisseroth Albert B. , Spitzer Gary , Spencer Verneeda , Spinolo Jorge A. , Dicke Karel A. , Giralt Sergio 
Published: February 2007

AbstractForty‐three patients received etoposide, cyclophosphamide, and fractionated total body irradiation before allogeneic marrow transplantation. Fifteen patients had chronic myelogenous leukemia in chronic phase or acute leukemia in first remission (standard risk) and twenty‐eight patients with more advanced disease (high risk). All patients received etoposide 1,500 mg/m2 intravenously on day –8, cyclophosphamide 60 mg/kg/day intravenously on days –7 and –6, and total body irradiation at 170 cGy twice a day on days –3, –2, and –1. During the first 100 days 12 high risk patients (43%) died from causes unrelated to relapse while none of the standard risk patients died. Renal and hepatic dysfunction were also significantly increased during the first 14 days in the high risk group. The addition of 1,500 mg/m2 of etoposide to the cyclophosphamide and total body irradiation was well tolerated for patients with standard risk. However, the regimen was poorly tolerated with high mortality in patients with more advanced disease. © 1992 Wiley‐Liss, Inc.
Elevated neutrophil function in chronic neutrophilic leukemia
Authors: Ohtsuki Tetsuya , Mizukami Hiroaki , Kimura Fumihiko , Ohnishi Mayumi , Nagata Naokazu , Motoyoshi Kazuo , Katsura Yoshiya , Matsu‐ura Yoshifusa 
Published: February 2007

AbstractA 65‐year‐old man with marked leukocytosis was admitted for diagnosis and treatment. His peripheral blood leukocyte count was 37,500/μI and the leukocytes consisted of mature neutrophil‐like cells. A high neutrophil alkaline phosphatase score and a normal bone marrow cell karyotype suggested that the patient had chronic neutrophilic leukemia rather than chronic myeloid leukemia. Several neutrophil functions, such as superoxide production, nitroblue tetrazolium reduction activity, and phagocytosis, were elevated. These data and the morphological features (toxic granules and Döhle bodies) indicated that the patient's neutrophils were in an activated stage. © 1992 Wiley‐Liss, Inc.
Role of endothelin in disseminated intravascular coagulation
Authors: Asakura H. , Jokaji H. , Uotani M. Saito C. , Kumabashiri I. , Morishita E. , Yamazaki M. , Matsuda T. 
Published: February 2007

AbstractWe examined the changes in plasma levels of endothelin‐1 (ET‐1), a potent vasoconstrictor peptide, in 47 cases of disseminated intravascular coagulation (DIC) to investigate the role of ET‐1 in DIC and its relation to multiple organ failure (MOF). A significant elevation of plasma levels of ET‐1 was observed in some cases of DIC, especially in patients with sepsis, blastic crisis of chronic myelogenous leukemia, and cancer. However, no such significant elevation was observed in patients with acute promyelocytic leukemia (APL), acute leukemias except for APL, or non‐Hodgkin lymphoma. Plasma levels of ET‐1 were higher in patients with DIC with MOF than in those without MOF. Although the levels of ET‐1 were decreased or remained low with clinical improvement in most DIC patients, the levels were further increased or remained high in patients who showed no improvement in DIC. It is suggested that ET‐1 must play an important role in further progression of MOF with the vasoconstriction and microcirculatory disorders. © 1992 Wiley‐Liss, Inc.
5‐Aminolevulinate synthase in sideroblastic anemias: mRNA and enzyme activity levels in bone marrow cells
Authors: Bottomley Sylvia S. , Brandenburg Mark A. , Healy Helen M. , May Brian K. 
Published: February 2007

AbstractTo examine the role of 5‐aminolevulinate synthase (ALAS) in the pathogenesis of sideroblastic anemias, levels of mRNAs for erythroid and housekeeping ALAS isozymes were examined, and total ALAS activity was assessed in bone marrow cells. In two patients with X‐linked sideroblastic anemia the levels of mRNA for erythroid ALAS as well as for α and β globin appear to be decreased while levels of mRNA for glycophorin A in both patients were the same as in normal individuals. However, amounts of housekeeping ALAS mRNA were increased two‐ to threefold in these patients. Total ALAS activity was also increased two‐ or threefold, perhaps reflecting increased transcription of the housekeeping gene in response to diminished cellular heme in erythroid cells and/or enhanced translation of the erythroid isoform in response to iron accumulation. In a third patient with X‐linked sideroblastic anemia ALAS activity was low but increased to twice the normal value after pyridoxine administration, suggesting a structural defect of the enzyme. In a fourth patient, with isolated congenital, pyridoxine‐responsive sideroblastic anemia, the erythroid ALAS mRNA was normal and a low enzyme activity was strikingly enhanced by pyridoxal‐phosphate albeit to subnormal levels. In idiopathic acquired sideroblastic anemia, ALAS mRNA for both isozymes was normal and enzyme activity was slightly elevated. These observations thus reflect heterogeneous aberrations of erythroid heme synthesis in the various types of sideroblastic anemia and suggest that defects involving erythroid ALAS underlie at least some of them. © 1992 Wiley‐Liss, Inc.
Procoagulant activity of endotoxin or tumor necrosis factor activated monocytes is enhanced by IgG from patients with lupus anticoagulant
Authors: Schved Jean‐Fraņois , Gris Jean‐Christophe , Ollivier ValéRie , Wautier Jean‐Luc , Tobelem GÉRard , Caen Jacques 
Published: February 2007

AbstractThe effect of lupus anticoagulant (LA) positive plasma on the expression of human monocyte procoagulant activity (PCA) was studied. LA positive plasma were able to enhance the endotoxin or TNF alpha induced monocyte associated PCA. The monocyte PCA had the characteristic of tissue factor activity (factor VII, factor × dependence). The enhancement of monocyte PCA could be confirmed using purified LA positive IgG. The stimulating effect was supported by the F(ab')2 fragments. © 1992 Wiley‐Liss, Inc.
Detecting of the minimal residual disease contaminated in peripheral blood stem cell transplantation in the B‐Cell malignant lymphoma patients
Authors: Nagasu Masaya , Aizawa Shin , Hojo Hitoshi , Tsuda Akemi , Yaguchi Makoto , Nakano Masaru , Toyama Keisuke 
Published: February 2007

AbstractFor sufficient collection of hemopoietic stem cells from peripheral blood for autologous peripheral blood stem cell transplantation (PBSCT), four patients with B‐cell‐type non‐Hodgkin lymphoma (B‐NHL) were examined for the appearance of circulating hemopoietic progenitors in blood (PSC) during the hemopoietic recovery phase following marrow ablative therapy in combination with or without administration of recombinant human granulocyte colony‐stimulating factor (rhG‐CSF). Each patient received only chemotherapy in the first course, and rhG‐CSF (1 βg/kg/day) was administered for 14 consecutive days from the last day of the second chemotherapy. In the second chemotherapy course with rhG‐CSF administration, white blood cell (WBC) counts demonstrated two peaks, and the appearance of granulocyte–macrophage precursor cells (CFU‐GM) in blood at the maximum level was coincident with the second peak of WBC elevation. Erythroid precursor cells (BFU‐E) were also detectable in blood after chemotherapy but the peak level was not enhanced by the use of rhG‐CSF. To determine whether the minimal residual disease (MRD) cells were contaminated in PSC corrected from blood, kappa–lambda imaging (KLI) analysis was performed to detect the malignant B‐cell population (mBp) before and after chemotherapy. No mBp was found in two of four patients in blood, although three of them were involved with mBp in bone marrow. The presence of mBp was detected in two patients both before and after chemotherapy, even though these cells were hardly detected morphologically, suggesting the necessity of judging for the incidence of contamination of MRD cells when collecting PSCs. © 1992 Wiley‐Liss, Inc.
Malignant tγ/δ lymphoproliferative disease with natural killer lytic activity
Authors: Falcão Roberto P. , Voltarelli Julio C. , Simões Belinda P. , Pestana Denise N. R. , Zago Marco A. , Figueiredo Mauro S. 
Published: February 2007

AbstractA 17‐year‐old girl presented with a lymphoproliferative disease involving the bone marrow, peripheral blood, and liver associated with reactive hyperplasia of the spleen. Neoplastic cells were atypical medium‐sized lymphoblasts with convoluted nuclei and nucleoli without features of large granular lymphocytes (LGL). The phenotype was CD3+CD4‐CD8‐, TCRα/β‐, TCRγ/δ+, δTCS1‐. and CD16+, and these cells exhibited spontaneous natural killer (NK) activity. DNA analysis showed rearrangement of the TCRγ gene but not of TCRβ or of lg μ genes. This unusual lymphoproliferative disease may represent the neoplastic expansion of a minor subset of normal Tγ/δ cells with NK activity. © 1992 Wiley‐Liss, Inc.
Accessory spleen in recurrent chronic immune thrombocytopenic purpura
Authors: Facon T. , Caulier M. T. , Fenaux P. , Plantier L. , Marchandise X. , Ribet M. , Jouet J. P. , Bauters F. 
Published: February 2007

AbstractFrom 1969 to 1985 we discovered accessory spleens in 8 patients with chronic immune thrombocytopenic purpura (ITP) who relapsed or failed after splenectomy. Imaging of accessory spleen used a liver spleen scintigraphy with heat‐treated RBC labeled with Tc‐99m. Platelet kinetic studies with 51Cr or 111In, including sequestration index, were performed. Five patients had accessory splenectomy. Disappearance of bleeding symptoms was achieved in the 5 splenectomized patients but with only partial response of platelet counts. These results are discussed in the context of diagnosis and therapeutic management of accessory spleens in patients with chronic immune thrombocytopenic purpura who relapsed or failed afler splenectomy. © 1992 Wiley‐Liss, Inc.
Molecular basis of beta thalassemia in the south of thailand
Authors: Laosombat V. , Fucharoen S‐P. , Panich V. , Fucharoen G. , Wongchanchailert M. , Sriroongrueng W. , Nopparatana C. , Kenpitak K. , Maipang M. , Fukumaki Y. 
Published: February 2007

AbstractA total of 103 beta thalassemia genes from 78 children (45 with Hb E/beta thalassemia, 8 with beta thalassemia heterozygotes, and 25 with homozygous beta thalassemia) were analyzed using dot‐blot hybridization of the polymerase chain reaction‐amplified DNA and direct DNA sequencing. Nine mutations were characterized in 98/103 (95%) of beta thalassemia alleles, of which six (a 4 bp deletion in codons 41–42, a G‐C transition at position 5 of IVS‐1, A‐G transition at codon 19, an A‐T transition at codon 17, an A‐G transition at position –28 upstream of the beta globin gene, a G‐T transition at position 1 of IVS‐1), accounted for 92%. The spectrum of beta thalassemia mutations in Chinese Thai is similar to that reported among the Chinese from other parts of the world. The distribution of beta thalassemia mutations in Muslim Thai is similar to that reported among Malaysians. The most common beta thalassemia mutation in Thai and Chinese Thai patients is the frameshift mutation at codons 41–42, in comparison with the Muslim Thai in whom the G‐C transition at position 5 of the IVS‐1 mutation predominates. The heterogeneity of molecular defects causing beta thalassemia should aid in the planning of a prenatal diagnosis program for beta thalassemia in the South of Thailand. © 1992 Wiley‐Liss, Inc.
Cytogenetic characterization of primary refractory anemia
Authors: Gyger Martin , d'Angelo Giovanni , Bélanger Robert , Forest Lorraine , Lussier Pauline , Busque Lambert , Perreault Claude , Boileau Jacques , Bonny Yvette , Lavallée René , Lacombe Michel , Roy Denis‐Claude 
Published: February 2007

AbstractRefractory anemia (RA) is the only myelodysplastic syndrome (MDS) devoid of quantitative marrow diagnostic criteria. The diagnosis rests mainly on the subjective identification of qualitative abnormalities according to the French‐American‐British criteria (FAB) involving one or more bone marrow hematopoietic cell lineages. The occurrence of nonrandom chromosome abnormalities remains the hallmark of the disease and the only means of investigation which confirms the disease objectively. With the purpose in mind to further characterize RA among MDS, we have undertaken a prospective high resolution banding chromosome analyses of bone marrow cells in patients with primary refractory anemia (PRA) with the aim of defining a cytogenetic phenotype and of assessing the clinical relevance of clonal abnormalities at initial diagnosis. Of 39 patients consecutively referred for chromosome analyses with a diagnosis of RA according to the FAB criteria, 27 patients had PRA and fulfilled our criteria for adequate chromosome analyses. Median age was 68 years. Fourteen of 27 patients (52%) had clonal chromosomal abnormalities at diagnosis. None of the patients showed a complex karyotype; 9/14 (64%) had a mixture of normal and abnormal cells. Interstitial or terminal deletions, involving chromosomes 5, 6, 7, 9, 11, 12, and 20, were found in 11/14 (79%) of the patients. Comparison of survival between patients with and without abnormalities showed no difference. The presence of clonal abnormalities did not predict transformation to acute myeloblastic leukemia (AML) nor was it associated with poor survival. In this study, patients with PRA were found to have a predominant pseudodiploid karyotypic pattern characterized by interstitial and/or terminal deletions as opposed to derivatives, specific and non‐specific balanced translocations, or other structural and numerical abnormalities. We were unable to reveal any prognostic significance to the presence of these clonal abnormalities at initial diagnosis. © 1992 Wiley‐Liss, Inc.
Clinical, hematological, and molecular features in sicilians with Hb S‐β‐thalassemia
Authors: Schiliro' G. , Samperi P. , Testa R. , Gupta R. B. , Gu L.‐H. , Huisman T. H. J. 
Published: February 2007

AbstractThe clinical, hematological, and molecular features of 81 patients with Hb S‐β‐thalassemia and relatives from 76 unrelated families are reported. We analyzed the β‐thalassemia mutations and the βS haplotypes in all patients and detected 6 different β‐thalassemia alleles: codon 39 (C→T) (39 cases), IVS‐I‐1 (G→A) (12 cases), IVS‐II‐1 (G→A) (4 cases), IVS‐l‐6 (T→C) (6 cases), IVS‐I‐110 (G→A) (14 cases), and IVS‐II‐745 (G→C) (6 cases). Eighty patients had haplotype #19 or the Benin type and one had haplotype #17 or the Cameroon type. The type of β‐thalassemia allele had the greatest influence on the phenotypic expression; this was observed for patients with Hb S‐β‐thalassemia and for simple β‐thalassemia heterozygotes. The mild IVS‐I‐6 (T→C) mutation produced borderline abnormal erythrocytic indices and Hb A2 levels in heterozygotes. Overall, there was a milder expression in βSβ+ patients (only 7.7% presented severe disease) than in those with the βSβ° condition (22.6% had the severe form of the disease). © 1992 Wiley‐Liss, Inc.
Adult T‐cell leukemia developing during immunosuppressive treatment in a renal transplant recipient
Authors: Tsurumi Hisashi , Tani Kenzaburo , Tsuruta Toshihisa , Shirato Risa , Matsudaira Tadahiro , Tojo Arinobu , Wada Chikashi , Uchida Hisanori , Ozawa Keiya , Asano Shigetaka 
Published: February 2007

AbstractWe report a case of a 32‐year‐old male, an asymptomatic carrier of human T‐cell leukemia virus type 1 (HTLV‐1), who underwent a renal transplantation and developed adult T‐cell leukemia (ATL) during the course of posttransplant immunosuppressive treatment. He was treated with combination chemotherapies consisting of cyclophosphamide, vincristine, doxorubicin, prednisolone, cisplatin, cytosine arabinoside, etoposide, and methyl‐prednisolone, without any improvement. Bestrabucil (KM2210), a conjugate of chlorambucil and estradiol, was administered as an alternative therapy; this therapy successfully suppressed his leukemic cell growth, and partial remission was achieved. Posttransplant immunosuppressive therapy with prednisolone, mizoribine, and cyclosporin A might have been the predominant cause of the transition from an asymptomatic HTLV‐1 infection to overt ATL. A careful approach is required with HTLV‐1 asymptomatic carriers who need organ transplantation followed by immunosuppressive treatment. © 1992 Wiley‐Liss, Inc.
Methemoglobinemia
Authors: Mansouri Ali , Lurie Aubrey A. 
Published: February 2007

AbstractThe ferrous iron of hemoglobin is exposed continuously to high concentrations of oxygen and, thereby, is oxidized slowly to methemoglobin, a protein unable to carry oxygen. To restore hemoglobin function, methemoglobin (ferrihemoglobin) must be reduced to hemoglobin (ferrohemoglobin). Under physiological conditions, methemoglobin reduction is accomplished mainly by red cell NADH‐cytochrome b5 reductase (NADH‐methemoglobin reductase) so efficiently that there is insignificant amounts of methemoglobin in the circulating blood. However, should methemoglobin formation be increased—e.g., due to the presence of oxidant drugs, or an abnormal methemoglobin not amenable to reduction (hemoglobin M), or a deficiency in red cell cytochrome b5 reductase—methemoglobinemia will result. Most methemoglobinemias have no adverse clinical consequences and need not be treated. Under certain conditions, such as exposure to large amounts of oxidant or in young infants, rapid treatment is necessary. In hereditary cytochrome b5 deficiency, treatment is often directed at improving the poor cosmetic effect of persistent cyanosis with the minimum amount of drugs to give satisfactory clinical results. © 1993 Wiley‐Liss, Inc.
Fetal hemoglobin reactivation in baboon and man: A short perspective
Authors: Lavelle Donald , Desimone Joseph , Heller Paul 
Published: February 2007

AbstractPresent concepts of the mechanism of reactivation of synthesis of fetal hemoglobin (HbF) in the adult under conditions of erythropoietic stress are briefly reviewed. Since HbF can be considered an effective natural antisickling agent, the reactivation of its synthesis in patients with sickle cell anemia as a desirable therapeutic goal has been extensively explored since the discovery in 1982 that 5‐azacytidine increases HbF levels in the baboon. Hydroxyurea (HU) has become the most widely used agent, although its effectiveness in increasing HbF levels and the number of F cells is highly variable. Recent investigations are cited showing that other agents such as butyrate, and the addition of recombinant hemopoietic growth factors, such as erythropoietin and stem cell factor, especially in combination with HU, offer important therapeutic possibilities. Transacting nuclear proteins are briefly discussed as possibly having a future role in the efforts of stimulating γ‐chain synthesis. © 1993 Wiley‐Liss, Inc.
Band 3 peptides inhibit deoxy S polymerization: Viscosity studies
Authors: Danish Elizabeth H. , Lundgren David W. , Harris John W. 
Published: February 2007

AbstractWe have previously obtained evidence that N‐terminal band 3 peptides inhibited deoxyhemoglobin S (deoxy S) polymerization as determined by equilibrium solubility assays. An N:1‐15AA fragment binds to the 2,3‐diphosphoglycerate (2,3‐DPG) receptor locus of deoxy S with five to seven amino acids (AA) extending internally, while ten to eight AA remained external to deoxy S and inhibited polymerization by steric hindrance. A true mirror‐image peptide, corresponding to two N:1‐8AA + lysine (K) linked by coupler, binds to the 2,3‐DPG loci of two deoxy S molecules, tethering them together to form “binary complexes” incapable of entering the polymer chains. The reduction in the concentration of deoxy S available for extended chain formation decreased polymerization. We now report time:viscosity profiles of the sol‐gel transformation of purified solutions of deoxy S with and without peptides and studies of the gel solidity at equilibrium. Samples with peptides had longer lag times than controls of similar deoxy S concentrations. The mirror‐image peptide was a more effective inhibitor than the N:1‐15AA peptide. When the mirror‐image peptide was present in peptide:hemoglobin molar ratios of 0.25‐1:1, the increases in lag time were equivalent to decreasing the deoxy S concentrations by 15–25%, comparable to projected major therapeutic effects. Gel solidity, determined by yield temperature, was less in the sample with mirror‐image peptide compared to control. These results support the proposed mechanisms of inhibition of deoxy S polymerization by band 3 peptides. © 1993 Wiley‐Liss, Inc.
Evaluation of bone marrow iron stores in anemia associated with chronic disease: A comparative study of serum and red cell ferritin
Authors: Balaban Edward P. , Sheehan Richard G. , Demian Saba E. , Cox John V. , Frenkel Eugene P. 
Published: February 2007

AbstractThe serum ferritin (SERFER) may be elevated and misleading in the setting of chronic disease (chronic inflammation, liver disease, and neoplasm). The red cell ferritin (RBCFER) may be more stable in clinical situations that affect the SERFER. We compared the ability of SERFER and RBCFER to assess iron stores in these settings. Iron stores were defined by bone marrow aspirate staining. We studied 120 anemic (Hb < 14 g/dl) male patients. Twenty‐eight (23%) were iron deficient based on the absence of marrow iron. The SERFER correlation with marrow iron stores (r = 0.58; P < 0.001) was better than the RBCFER (r = 0.36; P < 0.001). Cutoff values for the diagnosis of iron deficiency were determined by chi‐square analysis (SERFER < 70 ng/ml; RBCFER ± 4 ag/RBC). The sensitivity for detecting iron deficiency with SERFER (0.60) was less than RBCFER (0.82). The specificity of SERFER (0.90) was slightly better than RBCFER (0.83). Neither difference reached statistical significance (P > 0.05). The positive predictive value between the two assays was the same (SERFER 0.65 vs. RBCFER 0.59). The combination of SERFER <70 ng/ml with RBCFER ≤ 4 ag/RBC was more specific (0.97) when compared with the SERFER alone (0.90) (P = 0.04). In addition, the potential of this combination to predict iron deficiency (0.82) was higher than that seen with either SERFER (0.65) or the RBCFER (0.59). Our findings show that the RBCFER as a single assay is not anymore accurate than the SERFER. However, we find that the RBCFER can effectively complement the SERFER to either predict iron depletion or confirm the presence of bone marrow iron. © 1993 Wiley‐Liss, Inc.
Platelet activation in patients with thrombotic thrombocytopenic purpura
Authors: Hoffman Maureane , Monroe Dougald M. , Roberts Harold R. 
Published: February 2007

AbstractThrombotic thrombocytopenic purpura (TTP) is a rare syndrome of unknown etiology. It is characterized by platelet microthrombi in small vessels, which results in tissue dysfunction and a microangiopathic hemolytic anemia. Activation of coagulation is not a prominent feature of TTP. It is not known whether the process which results in platelet aggregate formation might also activate platelets. Using GMP‐140 as a marker of activation, we examined the activation state of circulating platelets in seven TTP patients and three normal controls, as well as the ability of purified platelets from three TTP patients and three controls to be activated in vitro. There was no statistically significant difference in the percentage of activated platelets circulating in patients and controls (4% vs. 2%). Both TTP and control platelets increased GMP‐140 expression and procoagulant activity after stimulation with thrombin or the calcium ionophore A23187. Thus, we conclude that TTP patients do not have a significantly increased proportion of circulating activated platelets, and their platelets can be activated normally by thrombin or a calcium ionophore. © 1993 Wiley‐Liss, Inc.