bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intravenous extended infusion of recombinant human soluble thrombomodulin prevented tissue factor‐induced disseminated intravascular coagulation in rats
Authors: Mohri Mitsunobu , Oka Michiko , Aoki Yoshikazu , Gonda Yasunori , Hirata Sigeto , Gomi Komakazu , Kiyota Takao , Sugihara Taisuke , Yamamoto Shuji , Ishida Torao , Maruyama Ikuro 
Published: February 2007

AbstractThis study demonstrated that intravenous infusion of recombinant human soluble thrombomodulin (rhs‐TM) could Inhibit disseminated Intravascuiar coagulation (DIC) caused by 4 hr infusion of tissue factor (TF) in rats. Extended infusion of TF reduced fibrinogen and platelet counts and elevated serum FDP level. Pretreatment and coinfusion of rhs‐TM could block changes of these DIC‐parameters without prolongation of APTT. Heparin, which is a potent anti‐DIC drug, could also inhibit these changes with extra prolongation of APTT and PT. Thus, these results suggest thrombomodulin prevent DIC less bleeding tendency than heparin. © 1994 Wiley‐Liss, Inc.
Prevalence of antithrombin deficiency in healthy blood donors: A cross‐sectional study
Authors: Wells Philip S. , Blajchman Morris A. , Henderson Penny , Wells Michael J. , Demers Christine , Bourque Ron , McAvoy Ann 
Published: February 2007

AbstractThe prevalence of antithrombin (AT) deficiency In the general population has been variously estimated to be between 0.05 and 5 per 1,000 in the population; 2,491 blood donors were screened In an attempt to clarify this Issue using plasma samples taken from the blood donor units. From this initial population, 122 individuals were identified as having plasma AT levels lower than 2 standard deviations below the normal mean. Twenty‐two samples had evidence that thrombin had been generated during blood collection and the remaining cohort of 100 blood donors were asked to return but only 59 complied. The data obtained from these 59 were compared with that from 51 age‐ and sex‐matched control blood donors. Both groups of subjects were assessed for previous evidence, or family history, of thrombotic events, as well as exposure to risk factors associated with the development of deep vein thrombosis (DVT). All had venous blood samples taken from which the supernatant plasma was Immediately removed and quick frozen for later assaying. Only 6 of the 59 subjects with initial low AT levels had repeat AT‐Xa levels below 0.80 units/ml (normal range 0.94 ± 0.14). Upon repeating the AT‐Xa determinations on new samples from these six Individuals, only three were found again to be low. One was found to have a type 3 AT deficiency (an Arg47Cys substitution). The other two with a low AT level had mean functional AT‐Xa levels of 0.61 and 0.71 units/ml, respectlvely, with correspondingly low AT:Ag levels consistent with a type 1 AT deficiency. Two of these three subjects has been in high risk situations without evidence of having developed DVT and none had evidence of venous reflux on Doppler venography. In addition, none had personal or family histories of previous thrombotic events. These present data Indicate that the prevalence of AT deficiency In our blood donor population is 2 per 1,000 (95% confidence intervals: 0.7‐6/1,000). © 1994 Wiley‐Liss, Inc.
Two different mutations in codon 68 are observed in Hb G‐Philadelphia heterozygotes
Authors: Molchanova T. P. , Pobedimskaya D. D. , Ye Z. , Huisman T. H. J. 
Published: February 2007

AbstractThrough sequencing of amplified DNA containing the appropriate α‐giobin genes we have identified the base substitution leading to the formation of Hb G‐Philadelphia [α6B(El7)Asn Lys]. Three subjects (∼25% Hb G) had an AAC AAA change at codon 68 of the α2‐globin gene; the chromosome with this mutation carried two α genes (αα). Six subjects (∼33% Hb G) had an AAC AAG change at the same codon of the α2α1 hybrid gene on a chromosome with the 3.7 kb deletion (‐α3.7). These results indicate two inde pendent mutations which likely occurred in different populations; Increase In the level of Hb G is primarily dependent upon the loss of one or more α‐globin genes. © 1994 Wiley‐Liss, Inc.
CD34 immunohistochemistry of bone marrow biopsies: Prognostic significance in primary myelodysplastic syndromes
Authors: Soligo Davide A. , Oriani Alessandro , Annaloro Claudio , Cortelezzi Agostino , Calori Rossella , Pozzoli Ermanno , Nosella Diego , Orazi Attilio , Deliliers Giorgio Lambertenghi 
Published: February 2007

AbstractBone marrow (BM) biopsies from 58 patients with primary myelodysplastic syndrome (MDS) were studied using QBEND10, a monocional antibody that recognizes the human progenitor CD34 antigen in routine aldehyde‐fixed paraffin‐embedded samples. FAB subtypes were RA (5 patients), RARS (9 patients), RAEB (20 patients), RAEBt (11 patients), CMML (3 patients). In addition, 10 MDS patients whose BM biopsies revealed heavy reticulum fibrosis were included. Neither the percentage of CD34+ cells nor the number of CD34+ aggregates (defined as clusters of 3 or more cells) correlated with the presence and morphology of abnormal localizations of immature precursors (ALIP). When all patients were considered, median survival was 69 months in those with less, and 25 months in patients with more than 1% CD34+ cells (P < 0.05). Median survival was 15 months in patients with CD34+ aggregates and 41 months in those without aggregates (P = 0.0017). When RAEB patients were considered median survival was 41 months in those with less than 1%, and 29 months in those with more than 1% CD34+ cells; the 4‐year survival chance was 45% in the former and 18.3% in the latter group. Therefore, CD34 positivity of more than 1% identifies a subset of RAEB patients with shorter life expectancy. In addition, leukemic transformation was observed in 11 of 35 patients (31%) with no CD34 aggregates, but in 14 of 23 patients (60%) with aggregates (P < 0.05). CD34 immunostaining, which can be easily performed on routinely prepared BM biopsies, was found to be a powerful prognostic tool for predicting survival and outcome in MDS. © 1994 Wiley‐Liss, Inc.
Pure red cell aplasia associated with angioimmunoblastic lymphadenopathy with dysproteinemia
Authors: Lynch James W. , Elfenbein Gerald J. , Noyes Ward D. , Braylan Raul C. , Gross Mary Ann , Weiner Roy S. 
Published: February 2007

AbstractAngioimmunoblastic lymphadenopathy with dysproteinemia (AILD) can best be described as a disorder of T‐cells resulting in amplification of the B‐cell response and clinical symptoms of lymphadenopathy, fever, hepatosplenomegaly, and a variety of blood abnormalities. Pure red cell aplasia (PRCA), an autoimmune disorder resulting in selective aplasia of the erythroid series, has only rarely been associated with AILD. Herein we report three cases of AILD and PRCA. Serum from one patient was available for study and contained a dose‐dependent inhibitor of the CFU‐E but not CFU‐GM cultures from normal bone marrow. This activity was found in the globulin fraction after ammonium sulfate precipitation. Patients with AILD are known to make antibodies to many autologous epitopes, and the most well‐characterized mechanism of PRCA involves antibodies to red cell precursors. Our serum data are consistent with the hypothesis that such an antibody existed in our patient. Aggressive treatment of these patients resulted in transient improvement in two; however, all three died without achieving a durable complete remission with two dying of infectious complications. © 1994 Wiley‐Liss, Inc.
Genotype of subjects with borderline hemoglobin A2 levels: Implication for, β‐thalassemia carrier screening
Authors: Galanello R. , Barella S. , Ideo A. , Gasperini D. , Rosatelli C. , Paderi L. , Paglietti E. , Sollaino C. , Perseu L. , Loi D. , Cao A. 
Published: February 2007

AbstractIn this study, we have defined by molecular analysis, the α, β, and δ globin genotype in a group of individuals with normal or thal‐like red cell indices but borderline hemoglobin (Hb)A2 levels, who were identified in a program for β‐thal carrier screening. In 37 of 125 individuals with borderline HbA2 levels, we detected a molecular defect in the β, in both the δ and the β, or in the α globin gene. Specifically seven of these subjects were carriers of the −101 C T mutation, ten of the IVSI nt6 T C mutation, 16 were double heterozygotes for δ and β thal, and two had the triple α globin gene and two the single α globin gene deletion. From these results, we may conclude that subjects with borderline HbA2, particularly when they marry a typical β‐thal carrier, should be extensively investigated in order not to miss heterozygous β‐thalassemia. © 1994 Wiley‐Liss, Inc.
Anticoagulation with anisindione in patients who are intolerant of warfarin
Authors: Grosset Alan B. M. , Allen Jill E. , Rodgers George M. 
Published: February 2007

AbstractPatients who require oral anticoagulation usually receive warfarin. We used an indanedione drug, anisindione, in two patients who were intolerant of warfarin but who needed long‐term oral anticoagulation. The use of this alternative oral anticoagulant is reviewed. © 1994 Wiley‐Liss, Inc.
Zinc‐induced sideroblastic anemia: Report of a case, review of the literature, and description of the hematologic syndrome
Authors: Fiske Darrell N. , McCoy Harry E. , Kitchens Craig S. 
Published: February 2007

AbstractZinc ingestion has become increasingly popular in the lay and food faddist population. Herein described by way of a case report and review of the 13 cases in the literature is the syndrome of severe anemia associated with excessive and prolonged intake of oral zinc. The syndrome is characterized by anemia, granulocytopenia, and bone marrow findings of vacuolated precursors and ringed sideroblasts. Serum analysis reveals increased zinc levels, decreased copper levels, and a decrease in ceruloplasmin. The mechanism appears to be zinc‐induced copper deficiency, which is instrumental in producing the profound bone marrow abnormalities, as zinc itself is of low toxicity. Importantly, the syndrome is totally reversible with cessation of zinc intake. Hematologists should be aware of this form of reversible sideroblastic anemia. © 1994 Wiley‐Liss, Inc.
Detection of common deletional α‐thalassemia‐2 determinants by PCR
Authors: Baysal E. , Huisman T. H. J. 
Published: February 2007

AbstractWe have developed a rapid approach to detect the two most common α‐thalassemia‐2 (α‐thal‐2) determinants by the polymerase chain reaction (PCR) technique, which takes a few hours to complete. Specific oligonucleotides selectively amplify appropriate segments of the chromosome with the deletion and the normal chromosome under identical experimental conditions, and the products are identified by electrophoresis on 1.5% agarose. Characterization of the two most prevalent types of the −α3.7 determinant [–α3.7(1) and −α3.7(11)] can be made by Apa I digestion of the PCR product. Two types of α‐thal‐2 determinants, −α3.7 and −α4.2, were tested in numerous samples from various parts of the world. This approach is believed to provide a cost‐effective way to screen large numbers of blood samples in a relatively short time and can be used to identify α‐thal‐2 heterozygotes and homozygotes and compound heterozygotes (–α3.7/–α4.2) in populations where such α‐gene defects are shown to exist at high frequencies. © 1994 Wiley‐Liss, Inc.
Familial myeloproliferative syndrome
Authors: Pérez‐Encinas M. , Bello J. L. , Pérez‐Crespo S. , De Miguel R. , Tome S. 
Published: February 2007

AbstractFamilial chronic myeloproliferative syndrome (CMS) was observed in five members from two different generations of the same kindred. Diagnoses included agnogenic myeloid metaplasia (case 1), polycythemia vera (case 2), and essential thrombocythemia (cases 3–5). Cases 1–3 were siblings, case 5 was the daughter of case 1, and case 4 was the cousin of cases 1, 3. Age at diagnosis ranged from 28 to 75 years, cases 1 and 3 were male, and the others were female. The diagnosis was made after an episode of cerebral thrombosis in one patient, during a study for headache and dizziness in another, and fortuituously in the three remainders. All patients had splenomegaly and varying degrees of thrombocytosis. The cytogenetic exam was normal in all four cases. A woman patient was treated with interferon during a pregnancy. Fetal growth was retarded, and the newborn showed bone and genital malformations. No environmental leukemogen factor was found. This familial case strengthens Dameshek's theory of a common pathogenesis of CMS and suggests a genetic and hereditary etiology. © 1994 Wiley‐Liss, Inc.
Relationship of burst‐forming‐unit‐erythroid progenitors and their DNA‐synthesis stage to fetal hemoglobin levels in hydroxyurea‐treated patients with sickle cell anemia
Authors: Mankad Vipul N. , Baliga Surendra , Phillips Karen , Shah Arvind K. , Yang Yih‐Ming 
Published: February 2007

AbstractDNA‐synthesis stage and total number of circulating burst‐forming‐units‐erythroid (BFU‐E) have been inversely correlated with hemoglobin F levels in the peripheral blood, as well as in the cells from the BFU‐E‐derived colonies obtained from homozygous sickle cell anemia (SS) patients during steady state. Similar studies in SS patients treated with cytotoxic agents have not been reported. However, regeneration of the erythroid marrow that follows the cytoreduction phase of chemotherapy has been suggested as one of the mechanisms of stimulation of fetal hemoglobin synthesis. Therefore, a longitudinal study of hemopoiesis in hydroxyurea‐treated SS patients was conducted. Thirty‐two sets of hemopoietic studies, including total circulating BFU‐E and S‐phase BFU‐E, were obtained from three patients treated with hydroxyurea. A dose‐dependent decrease in total BFU‐E colonies occurred in peripheral blood of all three patients (r = −0.58, −0.85, and −0.97, respectively, with each P < 0.05). There was a strong positive correlation between hydroxyurea dose and fetal hemoglobin levels in two of the three patients who responded clinically (r = 0.89618 and 0.88632, respectively, with each P < 0.01). When data from all patients were combined (n = 32), there was a strong, inverse, linear relationship between total number of BFU‐E and percentage S‐phase BFU‐E with fetal hemoglobin levels (r = −0.6649 and −0.7404, respectively, with each P < 0.0001). A stronger, curvilinear, multiple relationship was detected between total BFU‐E and percentage S‐phase BFU‐E with fetal hemoglobin levels (R = 0.8351 and 0.8602 with each P < 0.0001). © 1994 Wiley‐Liss, Inc.
Bepridil as an antisickling agent: Membrane internalization and cell rigidity
Authors: Johnson Robert M. , Acquaye Clemenceau , Féo Claude , Sarnaik Sharada 
Published: February 2007

AbstractThe calcium channel antagonist, bepridil, β‐(2‐methylpropoxy)methyl‐N‐phenyl‐N‐(phenylemethyl)‐1‐pyrrolidineethanamine monocholoride monohydrate, inhibits the sickling of deoxygenated sickle (SS) erythrocytes, as determined by light microscopy. The antisickling effect was seen only in dilute suspensions of red cells. In concentrated erythrocyte suspensions, sickling was not inhibited and measurements of hematocrit and cell density were unchanged by bepridil. The determination of cell volume in dilute suspensions was complicated by bepridil's tendency to aggregate, but rapid measurements by electronic sizing also indicated no increase in cell volume, up to a bepridil concentration of 200 μM. Ektacytometry of dilute sickle cell suspensions suggested an explanation for the anti‐sickling action of bepridil. Osmotic scan ektacytometry disclosed that bepridil initially increased the surface area of the red cell, as shown by a shift in the low osmolality minimum. This change was complete in 10 sec, the shortest time that could be measured. Subsequently, at concentrations that were observed to inhibit the sickling of deoxygenated sickle cells (100 μM or greater), red cells underwent a loss in surface area that was complete in 1 min. There was a concomitant loss of cell deformability. Light and scanning electron microscopy has previously shown that bepridil is a stomatocytic agent. Using transmission electron microscopy, we verified that the loss of surface area was a consequence of endocytosis, presumably as the end stage of the stomatocytic transformation induced by bepridil. Bepridil did not inhibit intracellular hemoglobin S polymerization even at 200 μM, as shown by oxygen scan ektacytometry. Bepridil thus appears to inhibit the sickling of deoxygenated SS cells by inducing endocytosis and lowering cell deformability. This mechanism may explain the anti‐sickling effect of other basic amphiphiles, such as chlorpromazine. © 1994 Wiley‐Liss, Inc.
Outcome of portal‐systemic shunt surgery for portal hypertension associated with intrahepatic obstruction in patients with agnogenic myeloid metaplasia
Authors: Tefferi Ayalew , Barrett Scott M. , Silverstein Murray N. , Nagorney David M. 
Published: February 2007

AbstractLife‐threatening portal hypertension (PHN) in patients with chronic myeloproliferative disorders may result from increased portal flow caused by marked splenomegaly or an increased resistance to portal flow from either a large vein thrombosis or an intrahepatic obstruction usually associated with agnogenic myeloid metaplasia (AMM). The former cause is correctable by splenectomy alone, whereas the latter requires portal‐systemic shunt surgery. Few data exist regarding the outcome of portal‐systemic shunt surgery in patients with AMM and intrahepatic obstruction. During the past 25 years, 13 patients with chronic myeloproliferative disorders underwent portal‐systemic shunt surgery at our institution. The cause of PHN was intrahepatic obstruction in ten patients and hepatic vein thrombosis in three. Ten of the thirteen patients had AMM as initial diagnosis. Only one patient had intraoperative complications, and four patients had either sepsis or thrombosis during the postoperative period. Twelve patients survived the postoperative period and had a median postsurgical survival of 3 years (range, 0.25 to 19 years). The long‐term complications of the operation were very few and included hepatic encephalopathy (one patient), portal vein thrombosis (one patient), and shunt occlusion (one patient). The procedure was successful in alleviating complications of PHN in all but one patient. Deterioration of hepatic function and subsequent hepatomegaly were unusual. Portal‐systemic shunt surgery seems to be a useful option in patients with AMM and lifethreatening PHN from intrahepatic obstruction. © 1994 Wiley‐Liss, Inc.
β‐thalassemia alleles and unstable hemoglobin types among russian pediatric patients
Authors: A.çürük M. , Molchanova T. P. , Postnikov Yu. V. , Pobedimskaya D. D. , Liang R. , Baysal E. , Kolodey S. , Smetanina N. S. , Tokarev Yu. N. , Rumyantsev A. G. , Huisman T. H. J. 
Published: February 2007

AbstractA recently initiated collaboration between Russian and American institutions has resulted in the characterization of several known or new β‐thalassemia alleles and unstable hemoglobin types. Nine known β‐thalassemia alleles were present which have also been found in Mediterranean, East Asian, and Black populations; the possibility of independent mutations for some of the rare alleles should be considered. Hb Durham‐N.C./Brescia with a codon 114 (CTG → CCG; Leu → Pro) change was present in six members of two families. This condition and two new variants have the characteristics of a dominant type of β‐thalassemia heterozygosity with moderate anemia, Heinz body formation, splenomegaly, etc. One new β‐thalassemia allele is a frame‐shift at codon 124 (‐A), while another is characterized by the introduction of an extra proline residue (codon: CCA) between residues Thr (β123) and Val (β126) to give the sequence ‐Thr‐Pro‐Pro‐Pro‐Val‐. © 1994 Wiley‐Liss, Inc.
Congenital afibrinogenemia
Authors: Al‐Mondhiry Hamid , Ehmann W. Christopher 
Published: February 2007

AbstractCongenital afibrinogenemia is a rare disorder with unusual clinical manifestations. The disease is inherited as an autosomal recessive trait and consanguinity is common among affected families. Clinical manifestations range from minimal bleeding to catastrophic hemorrhage. Congenitally afibrinogenemic patients seem to be peculiarly susceptible to spontaneous rupture of the spleen. Coagulation tests which depend on clot formation as an end point may be infinitely prolonged and abnormalities of platelet function are usually present. The diagnosis is established by demonstrating trace or no immunoreactive fibrinogen. The disease is caused by markedly reduced or absent synthesis of fibrinogen by liver cells, but the genetic defect remains unknown. Bleeding episodes can be effectively treated with cryoprecipitate. Purified virally inactivated fibrinogen concentrates have been used in Europe and may soon be widely available. © 1994 Wiley‐Liss, Inc.
Regulation of erythropoietin production in a case of congenital erythropoietin‐dependent pure erythrocytosis
Authors: Borgna‐Pignatti Caterina , Liberato Nicola Lucio , Marradi Pierluigi , Rosti Vittorio , Barosi Giovanni 
Published: February 2007

AbstractIn a patient with congenital erythopoietin‐dependent pure erythrocytosis (EDPE) associated with hypersensitivity of erythroid progenitor cells to erythropoietin (Epo), the investigations planned to elucidate the mechanism responsible for hormone hyperproduction revealed that Epo synthesis was (1) independent of normal oxygen‐mediated feedback induced by phlebotomy; (2) not modulated by adenosine as a second messenger (the treatment with the adenosine antagonist theophylline in fact left unchanged the serum Epo levels); and (3) uninfluenced by iron therapy. The Epo dose—response curve for growth of erythroid progenitor was similar to that of three age‐matched thalassemia patients with increased serum Epo levels, (sEpo) suggesting that the observed erythroid progenitors hypersensitivity to Epo could represent an ex vivo artifact induced by the increased sEpo levels. © 1994 Wiley‐Liss, Inc.
Hemolytic anemia and acute renal failure associated with temafloxacin‐dependent antibodies
Authors: Maguire Robert B. , Stroncek David F. , Gale Eric , Yearlsey Martha 
Published: February 2007

AbstractQuinine‐ingestion has been associated with immune‐mediated recurrent pancytopenia, hemolysis, and renal failure. The structure of fluoroquinolone antibiotics is similar to the structure of quinine. Over a 3 month period, three patients at our institution developed hemolysis and renal failure following ingestion of the fluoroquinolone antibiotic temafloxacin. Two of the three patients required hemodialysis. Following withdrawal from the drug, the hemolysis resolved and the renal function eventually returned to normal in all three patients. One patient also had a transient mild thrombocytopenia. Sera from all three patients were tested for drug‐dependent antibodies to red blood cells, platelets, and neutrophils. Temafloxacin‐dependent red cell antibodies were detected in one patient, and temafloxacin‐dependent red cell and neutrophil antibodies were detected in a second patient. No temafloxacin‐dependent antibodies were detected in the third patient. Sera from all three patients were also tested for quinine and quinidine‐dependent antibodies to red cells, platelets, and neutrophils. Sera from the patient without temafloxacin‐dependent red cell antibodies reacted with red cells in the presence of quinine. These results suggest that, at least in some patients, the toxicities associated with temafloxacin are immune mediated. © 1994 Wiley‐Liss, Inc.