bluuebunny/crossref_metadata_2025_split huggingface dataset:
Elevated levels of heme oxygenase‐1 activity and mRNA in peripheral blood adherent cells of acquired immunodeficiency syndrome patients
Authors: Levere Richard D. , Staudinger Robert , Loewy Gabriel , Kappas Attallah , Shibahara Shigeki , Abraham Nader G. 
Published: February 2007

AbstractPatients with the acquired immunodeficiency syndrome (AIDS) commonly develop hematological abnormalities, including anemia, leukopenia, and thrombocytopenia. Heme synthesis and heme degradation are critical to the maintenance of cellular heme homeostasis and to hematopoietic differentiation. We examined heme oxygenase activity and expression of the heme oxygenase gene in adherent cells (monocytes‐macrophages) obtained from the peripheral blood of AIDS patients and normal controls. Heme oxygenase activity in normal control cells was 43 ± 16 pmol bilirubin formed/4 × 105 cells/hr as compared to 133 ± 30 pmol bilirubin formed/4 × 105 cells/hr in the AIDS patients. Via blot hybridization analysis with human heme oxygenase cDNA, heme oxygenase mRNA levels in cells of the normal and the AIDS patients were compared. Total RNA from normal cells displayed only weak hybridization with the cDNA probe. In contrast, cells from peripheral blood of the AIDS patients displayed marked increases over normal levels in heme oxygenase mRNA. Heme oxygenase activity could be substantially suppressed by the competitive inhibitor of the enzyme, Sn‐mesoporphyrin. Elevated heme oxygenase activity in cells of AIDS patients could produce a decrease in cellular heme needed for transductional signalling for the growth factor network, which regulates the hematopoietic microenvironment, and for other metabolic purposes. Suppression of heme catabolism by inhibitors of this enzyme may thus be useful in potentiating erythropoietic responses in this disorder.
Detection of immunoglobulin gene rearrangement in lymphoid malignancies of B‐cell lineage by seminested polymerase chain reaction gene amplification
Authors: Liang Raymond , Chan Vivian , Chan T. K. , Wong Thomas , Chiu Edmond , Lie Albert , Todd David 
Published: February 2007

AbstractSeminested polymerase chain reaction (PCR) was used to amplify the DNA fragments of the complementarity‐determining region 3 of the immunoglobulin (Ig) gene heavy chain from the malignant cell specimens of patients with leukemias and lymphomas of B‐cell lineage. Two different pairs of primers were used sequentially. Twenty of the 27 (74%) acute lymphoblastic leukemia (ALL) patients, 14 of 19 (74%) chronic lymphocytic leukemia (CLL) patients and eight of 20 (40%) non‐Hodgkin's lymphoma (NHL) patients, who had rearrangement of the Ig gene heavy chain by Southern analysis, were positive by the seminested PCR. False‐negative results appeared to occur more commonly in cases of lymphoma. The PCR analysis was also less likely to be positive if one‐stage PCR studies with either pair of primers were both negative. The seminested PCR technique was found to have a high sensitivity of detecting malignant cells at the level of 0.02%. The clinical application of this assay needs to be investigated further.
Linkage analysis of the hemoglobin F determinant(s) in an australian hemoglobin lepore (Boston) kindred
Authors: Motum Penelope I. , Donald Jennifer A. , Trent Ronald J. 
Published: February 2007

AbstractGenetic determinants that influence the levels of fetal hemoglobin (Hb F) in a single Australian kindred with heterozygous Hb Lepore (Boston) were sought. There were 22 affected individuals, some of whom had high Hb F and others with Hb F levels within the normal range. Family members were typed for restriction fragment length polymorphisms (RFLPs) associated with the β‐globin gene complex and the nearby genetic markers D11S12, INS, HRAS, and PTH. Prior to linkage analysis, a cohort of 54 unrelated Hb Lepore heterozygotes was analyzed to establish the distribution of Hb F levels measured by alkaline denaturation (Hb FAD). An Hb F level of >2.0% was used as the cutoff point for linkage analysis of the putative hereditary persistence of fetal hemoglobin (HPFH) determinant(s) in this kindred. Positive peak lod scores were obtained for the entire pedigree between the HPFH determinant and Hb Lepore (Zm+1 = 2.35 at θ = 0.15) and the β‐globin cluster (HBBC) (Zm+1 = 2.38 at θ = 0.20) marker loci, indicating the possibility of an independent HPFH gene at some distance from the β‐globin gene cluster. However, most of these lod scores result from the non‐Hb Lepore members of the family who, with one exception, do not have high Hb F, and when only those affected with Hb Lepore were analyzed the lod score values at these loci fell to small positive values (<1.0). These data do not support an independent cosegregating HPFH determinant separate from the Hb Lepore locus in this pedigree. The results favor a pleiotropic effect of the Hb Lepore lesion itself influencing Hb F levels by genetic or environmental factors not yet elucidated.
Chronic lymphocytic leukemia associated with bone marrow fibrosis: Possible role of interleukin 1α in the pathogenesis
Authors: Kimura Akiro , Hyodo Hideo , Nakata Yoshio , Kuramoto Atsushi 
Published: February 2007

AbstractB‐cell chronic lymphocytic leukemia (B‐CLL) associated with bone marrow fibrosis is described. The conditioned medium of the CLL cells contained a high quantity of interleukin 1α (IL‐1α), and showed growth promoting activity for marrow fibroblasts which was partially inhibited by anti IL‐1α antibody. In addition, the conditioned medium as well as IL‐1α stimulated the growth of marrow fibroblasts from the patient. These results strongly suggested the marrow fibrosis occurred by the secretion of IL‐1α from the CLL cells and its growth stimulation of marrow fibroblasts. As far as we know, this is the first case in which B‐CLL was associated with marrow fibrosis, and its mechanism was investigated.
Polycythemia associated with primary systemic amyloidosis: Elevated levels of hemopoietic factors and cytokines
Authors: Nagasawa Toshiro , Yanagisawa Hiromi , Hasegawa Yuichi , Kanma Hiroshi , Abe Tsukasa 
Published: February 2007

AbstractA 60‐year‐old man was diagnosed to have primary systemic amyloidosis (AL type) and polycythemia (Hgb 19.1 g/dl, WBC 14,600/μl, and platelets 60.5 × 104/μl). In vitro colony assay for hemopoietic stem cells showed increased numbers of colony‐forming units (CFU)‐G, CFU‐GM, CFU‐E, and CFU‐Meg. Serum levels of erythropoietin (Epo), granulocyte colony‐stimulating factor (G‐CSF), interleukin (IL)‐3 and IL‐6 were elevated. The infiltration of amyloid fibrils into the hemopoietic factor‐producing tissues may stimulate abnormal secretion of hemopoietic factors and cytokines, resulting in increased numbers of hemopoietic stem cells and polycythemia in the peripheral blood.
Coagulation system activation and increase of D‐dimer levels in peripheral arterial occlusive disease
Authors: De Buyzere M. , Philippé J. , Duprez D. , Baele G. , Clement D. L. 
Published: February 2007

AbstractThe aim of the present study was to document coagulation system activation and basal fibrinolysis in peripheral arterial occlusive disease (PAOD) at stage II of Fontaine's classification. In 34 patients, prothrombin fragment (F1 + 2), thrombin‐antithrombin III complexes (TAT), and D‐dimer concentrations were evaluated before and after a standard treadmill test. Basal levels in PAOD of F1 + 2 (1.25 ± 0.19 nmol/liter) and of TAT (3.34 ± 0.35 μg/liter) were significantly increased compared to those obtained in age‐ and sex‐matched healthy controls (0.68 ± 0.06 nmol/liter and 2.30 ± 0.33 μg/liter, respectively), showing baseline activation of the clotting cascade. A secondary activation of the fibrinolytic system was evidenced by the highly significant increase of basal D‐dimers (719 ± 99 ng/dl in PAOD vs. 229 ± 37 ng/dl in controls). Treadmill exercise failed to increase the study parameters significantly further. Walking distance (583 ± 40 m) was correlated with the preexercise ankle to brachial systolic blood pressure ratio (r = 0.485, P < 0.005) and inversely with the level of D‐dimers (r = −0.425, P < 0.02). Under baseline conditions, the latter parameter was correlated as well with the antigen concentration of urokinase‐type plasminogen activator (u‐PA; r = 0.503, P < 0.002). These results indicate that stage II PAOD is characterized by an activation of the clotting cascade in baseline conditions evidenced by increased F1 + 2 and TAT. A secondary activation of the fibrinolytic system with increased u‐PA antigen levels accounts for the elevated D‐dimers. Treadmill exercise was unable to increase these parameters further.
Comparison of buffy coat preparation to direct method for the evaluation and interpretation of bone marrow aspirates
Authors: Izadi Parvin , Ortega Jorge A. , Coates Thomas D. 
Published: February 2007

AbstractBone marrow differential and French, American, British (FAB) classification of buffy coat preparation (BCP) was compared to direct method (DM) in 69 pediatric patients with various hematologic and oncologic disorders. The marrow evaluation differed significantly in 12 of 69 patients (17.4%). The differential counts were discordant in 9 out of 69 patients (13%), and the FAB classifications were discordant in 3 out of 25 patients (12%) with acute lymphoblastic leukemia (ALL). Underestimation of the percent blasts occurred with buffy coat preparation in patients with acute non‐lymphoblastic leukemia (ANLL). While buffy coat preparations can facilitate morphologic evaluation in marrow malignancies, significant errors can occur in determination of the differential count. Direct smear should be used in conjunction with buffy coat smears in the evaluation of bone marrow aspirates.
DNA diagnosis for the detection of sickle hemoglobinopathies
Authors: Steinberg Martin H. 
Published: February 2007

AbstractAt this time, the sole generally accepted use for DNA diagnosis in the hemoglobinopathies is for the prenatal detection of disease, which can be identified by these means early in the first trimester of pregnancy. By ascertaining genotype rather than phenotype, the confusion that results from diagnostic errors should be diminished. DNA diagnostics are the future of all genetic disease detection and this future will soon be upon us.
Lymphoproliferative disease of 'lak cell' precursor large granular lymphocytes in association with celiac disease
Authors: Lopez P. , Morris D. G. , Galbraith P. R. , Lillicrap D. P. , Pross H. F. 
Published: February 2007

AbstractWe have investigated a case of lymphoproliferative disease of large granular lymphocytes (LDGL) occurring in association with celiac disease, anemia, neutropenia, and carcinomas of the endometrium, breast, and skin. The large granular lymphocyte (LGL) proliferation was monoclonal, T cell in origin, with T cell receptor β‐chain gene rearrangement, and a CD3+, CD8+, CD16± phenotype. In spite of the high frequency of LGL, natural killer (NK) cell activity was absent. Stimulation with interleukin‐2 in vitro, however, resulted in high lymphokine‐activated killer (LAK) cell activity against NK‐resistant targets. The T‐cell nature of the LAK precursor cells is in contrast to the majority seen in normal peripheral blood. Therapeutic trials of cyclosporin A, low‐dose cyclophosphamide, and levamisole were unsuccessful in reducing transfusion requirements. This case is unique in the association of LDGL with celiac disease. It is also unique in that the patient had been followed for several years prior to the onset of the LDGL. The case extends the list of lymphoproliferative disorders documented to be associated with celiac disease and, conversely, adds to our knowledge of lymphoproliferative disorder of LGL and its 'dysimmune' manifestations.
Chronic myelogenous leukemia and acute lymphoblastic leukemia occurring in the course of polycythemia vera
Authors: Roth Arnaud D. , Oral Arsinur , Przepiorka Donna , Gollin Susanne M. , Chervenick Paul A. 
Published: February 2007

AbstractWe are reporting an unusual case of a 54‐year‐old woman with polycythemia vera (PV) who developed Ph chromosome positive chronic myelogenous leukemia (CML) 8 years after the initial diagnosis of PV, and terminating in acute lymphoblastic leukemia (ALL), 11 years after the initial diagnosis. Cytogenetic studies revealed a normal female karyotype at the time of diagnosis of PV and the presence of a Ph chromosome at the time of appearance of CML. Southern blot hybridization revealed a bcr rearrangement in both mononuclear cells and granulocytes. The diagnosis of ALL was established on the basis of morphology, positive TdT staining, and monoclonal antibody studies positive for I2, B4, and J5.This case demonstrates the transition of PV into CML, followed by a blastic transformation into acute lymphocytic leukemia. At termination of her disease there were findings compatible with bi‐phenotypic leukemia. These findings would suggest that the disease arose in a primitive multipotential stem cell.
Hemostatic study before onset of disseminated intravascular coagulation
Authors: Wada Hideo , Minamikawa Kouzou , Wakita Yoshihiro , Nakase Tutomu , Kaneko Toshihiro , Ohiwa Michiaki , Tamaki Shigehisa , Deguchi Akira , Mori Yoshitaka , Deguchi Katsumi , Shirakawa Shigeru , Suzuki Koji 
Published: February 2007

AbstractEarly diagnosis is necessary for the treatment of disseminated intravascular coagulation (DIC), but criteria for the stage preceding the diagnosis of DIC (pre‐DIC) have not yet been established. To clarify hemostatic abnormalities that occur before the onset of DIC, we performed hemostatic studies in 117 patients within at least a week before the onset of DIC (pre‐DIC), in 237 patients with DIC, and in 50 patients without DIC or pre‐DIC (non‐DIC). Levels of FDP, PT, and fibrinogen, and platelet counts were significantly abnormal after the onset of DIC, but not before. Thrombin‐antithrombin III complex (TAT), plasmin‐α2 plasmin inhibitor complex (PIC), and FDP‐D‐dimer levels were significantly higher before the onset of DIC compared to the non‐DIC patients.Hemostatic abnormalities were observed within a week before the onset of DIC. Monitoring the plasma levels of TAT, PIC, and FDP‐D‐dimer might be useful for the diagnosis of a pre‐DIC condition.
Comparison between interphase and metaphase cytogenetics in detecting chromosome 7 defects in hematological neoplasias
Authors: Zhao Lian , van Oort June , Cork Ann , Liang Jan C. 
Published: February 2007

AbstractMonosomy 7 (–7) is one of the most common chromosomal abnormalities found in the leukemic cells of patients with acute myelogenous leukemia (AML) and myelodysplastic syndrome (MDS). Because patients with –7 have a poor prognosis, their identification is important for treatment planning. Conventionally, –7 is detected by the G‐banding technique. This study examines the use of fluorescent in situ hybridization (FISH) methodology to detect –7 cells in interphase nuclei and metaphase chromosomes. Fifteen AML or MDS patients whose leukemic cells were found to have –7 by G‐banding at disease presentation were studied. In 13 of these patients, –7 could be detected in interphase by FISH using a chromosome 7‐specific centromeric DNA probe. The two patients whose leukemic cells were not detectable by interphase FISH had –7 and t(1q;7p), which were detectable by FISH in metaphase using a chromosome 7‐specific painting probe. Metaphase FISH was particularly useful in further defining chromosome 7 defects in cells that contained aberrant or marker chromosomes. For example, in 6 patients, chromosome 7 sequences were detectable in aberrant or marker chromosomes by metaphase FISH, but not by G‐banding. These results suggest that metaphase FISH is an important adjunct to conventional cytogenetic methods for defining chromosome 7 abnormalities in AML and MDS patients. Furthermore, interphase FISH is useful for follow‐up studies in patients who are found informative for the FISH study at presentation.
Cell‐mediated immune‐pancytopenia complicating primary sjögren's syndrome
Authors: Seki Takeshi , Kiyosawa Kendo , Monno Satoshi , Imai Yasuharu , Furukawa Kenichi , Kumazawa Shigeyuki , Ota Masao , Saito Hiroshi , Furuta Seiichi , Nakahata Tatsutoshi 
Published: February 2007

AbstractA 64‐year‐old woman with mild bilateral parotid gland swelling and bilateral lower extremity purpura was admitted for evaluation of xerostomia and pancytopenia. The patient had an increased erythrocyte sedimentation rate, pancytopenia, and positive tests for antibodies to nuclear antigen, SS‐A, and SS‐B. Impaired cell‐mediated immunity was also present. Bone marrow aspiration showed a hypoplastic marrow with an increased percentage of lymphocytes. A positive Schirmer's test and keratoconjunctivitis were also noted. A diagnosis of primary Sjögren's syndrome was made by sialography and histological salivary gland findings. Therapy with prednisolone improved the pancytopenia. Addition of the patient's peripheral blood mononuclear cells to cultures of bone marrow mononuclear cells derived from a healthy volunteer dose dependently inhibited colony formation, including mixed hemopoietic colonies. On the other hand, addition of the patient's serum failed to inhibit colony formation by normal bone marrow mononuclear cells. These results suggested that one of the causes of pancytopenia in primary Sjögren's syndrome might be mediated by the inhibition of mononuclear cells to the hemopoietic progenitors.
Measurement of red blood cell‐vitamin B12: A study of the correlation between intracellular B12 content and concentrations of plasma holotranscobalamin II
Authors: Tisman G. , Vu T. , Amin J. , Luszko G. , Brenner M. , Ramos M. , Flener V. , Cordts V. , Bateman R. , Malkin S. , Browder T. 
Published: February 2007

AbstractWe have recently reported a new and rapid assay to measure plasma holotranscobalamin II (holo TC II) as a means of exploring vitamin B12 status. In order to further evaluate the significance of plasma holo TC II in determining tissue cobalamin, we have chosen the red blood cell‐vitamin B12 (RBC‐B12) assay as a measure of tissue vitamin B12 content and studied the relationship between RBC‐B12 and plasma holo TC II levels. Plasma holo TC II and RBC‐B12 concentrations were concomitantly assayed in 20 hematologically normal controls and cancer patients. In our groups of controls, the mean value of RBC‐B12 was determined as 241 ± 51 pg/ml of packed erythrocytes (PE) with a range varying from 180 to 355 pg/ml PE. Preliminary results obtained in 32 cancer patients revealed lower holo TC II and RBC‐B12 levels than the control group and a required threshold value of 70 pg/ml of holo TC II in order to maintain a normal RBC‐B12 greater than 180 pg/ml PE.
Mechanism of autoimmune hemolytic anemia in chronic lymphocytic leukemia
Authors: Sthoeger Zev M. , Sthoeger Dalia , Shtalrid Mordechai , Sigler Erica , Geltner David , Berrebi Alain 
Published: February 2007

AbstractChronic lymphocytic leukemia (CLL) is a malignant clonal expansion of CD5+B lymphocytes. The CD5+B lymphocytes have been postulated to produce autoantibodies. CLL patients may demonstrate features of autoimmunity including autoimmune hemolytic anemia. However, the origin of the autoantibodies causing the hemolysis is not clear. The present studies were performed to determine whether these autoantibodies are the products of the neoplastic B‐CLL clones. Immunoglobulins (Ig) were eluted from washed red blood cells (RBC) obtained from two CLL patients at the time they had autoimmune (DAT‐direct antiglobulin test ‐ positive) hemolytic anemia. The light chain phenotypes of these eluted autoantibodies were determined and found to be monotypic with exact correlation to the light chain expressed on the surface of the B‐CLL clones. Elutions from RBC of DAT negative patients or normal volunteers failed to demonstrate measurable amounts of Ig. In contrast, Ig eluted from RBC obtained from SLE patients with DAT positive hemolytic anemia found to be polyclonal autoantibodies exhibiting both light chain types. Furthermore, CD5+B lymphocytes obtained from the same two CLL patients (DAT+) produce, in vitro understimulation with phorbal myristate acetate (PMA), monoclonal antibodies which react and bind to RBC. Thus these studies provide direct evidence demonstrating that the antibodies causing the autoimmune hemolytic anemia in our two CLL patients are the products of the B‐CLL neoplastic clones.
Mitoxantrone and 5‐azacytidine for refractory/relapsed ANLL or CML in blast crisis: A leukemia intergroup study
Authors: Goldberg Jack , Gryn Jeff , Raza Azra , Bennett John , Browman George , Bryant John , Grunwald Hans , Larson Richard , Vogler Ralph , Preisler Harvey 
Published: February 2007

AbstractIn an effort to determine if cell cycle active agents are augmented when given after non‐cell cycle active agents, 104 patients with either multiply relapsed or refractory acute nonlymphocytic leukemia or blast crisis of chronic myelogenous leukemia were treated with mitoxantrone. Patients whose bone marrow did not show significant cytoreduction received 5‐azacytidine. Twenty‐seven of the 93 evaluable patients (23%) with ANLL achieved a complete remission. A total of 28% of patients receiving mitoxantrone alone achieved remission compared to 15% for those receiving mitoxantrone and 5‐azacytidine. Relapsed patients had a higher CR rate (36%) than refractory patients (15%). Nausea, vomiting, and stomatitis were common but rarely severe. The median duration of remission was 3.7 months and patients with abnormal karyotypes had longer remission durations than those with normal karyotypes. In this patient population, there was no evidence that 5‐azacytidine given after mitoxantrone increased the complete remission rate.
Sequential administration of cyclophosphamide and granulocyte‐colony stimulating factor relieves impaired myeloid maturation in felty's syndrome
Authors: Pixley John S. , Yoneda Ken Y. , Manalo Pacita B. 
Published: February 2007

AbstractA patient with Felty's syndrome (FS) and persistent profound neutropenia developed recurrent infections and sepsis syndrome. No impairment of granulocyte‐macrophage colony development was observed in vitro. Marrow morphology revealed an absence of mature neutrophil forms despite administration of granulocyte‐colony stimulating factor (G‐CSF). However, pretreatment with bolus cyclophosphamide (CY) permitted the growth factor to relieve this impairment of late myeloid maturation and resulted in a brisk, albeit short, burst of neutrophilia. This suggests that immune interference in myelopoiesis can be overcome by growth factor administration if immune activity is adequately dampened by immunosuppressive therapy.
Direct demonstration that the AγT globin gene is linked to the 4 bp promoter deletion in the βA chromosome of sickle cell traits
Authors: Gilman John G. , Josifovska Olgica , Erlingsson Soili , Nagel Ronald L. , Milner Paul F. 
Published: February 2007

AbstractIn βP°‐thalassemia and sickle cell patients, a 4 bp deletion at –222 to –225 of the Aγ globin promoter was associated with low expression of the AγT variant (threonine at codon 75 of Aγ), whereas AγI (isoleucine at 75) had the normal Aγ promoter and higher expression. However, it has been reported that the βA chromosomes of sickle cell trait cases have the 4 bp deletion as a common polymorphism unlinked lo the AγT allele. We now present data demonstrating the association of the AγT allele with the 4 bp deletion in βA chromosomes of sickle cell traits.
Familial lupoid thrombocytopenia
Authors: Shimizu Kazuyuki , Katsuta Itsuro , Uchikawa Takashi 
Published: February 2007

AbstractFamilial lupoid thrombocytopenia affecting three subjects in three generations is reported. All subjects had antinuclear antibodies with or without antibodies to single‐stranded DNA, an increase in platelet‐associated IgG, and a family member with an autoimmune disease. These findings implicate a genetic contribution. All subjects shared a common HLA haplotype. © 1993 Wiley‐Liss, Inc.