bluuebunny/crossref_metadata_2025_split huggingface dataset:
Unique sensitivity of Hb Zürich to oxidative injury by phenazopyridine: Reversal of the effects by elevating carboxyhemoglobin levels in vivo and in vitro
Authors: Virshup David M. , Zinkham William H. , Sirota Ronald L. , Caughey Winslow S. 
Published: February 2007

AbstractPhenazopyridirie (PAP) causes a hemolytic anemia in normal individuals who receive an overdose or in patients with decreased renal function given therapeutic doses. There are no reports of PAP‐induced hemolysis in individuals with unstable hemoglobins. Therapeutic doses of PAP administered to a subject with Hb Zürich (His E7 (63) β→ Arg) caused a severe hemolytic anemia with many large Heinz bodies appearing in the red cells.Incubation of whole blood from three asymptomatic Hb Zürich subjects with PAP at a molar ratio of PAP/Hb of 1.3/1 produced a moderate to marked increase in methemoglobin (MHb) and Heinz body formation accompanied by a slight to moderate decrease in levels of reduced glutathione. The rates of MHb formation were proportional to the concentration of PAP. In two of the subjects the rates of formation of Heinz bodies and MHb were substantially higher than in the third subject. Red cells from five normal adults were not affected. In Hb Zürich red cells the affinity of the abnormal β chain for carbon monoxide is much greater than that of the normal α and β chains. The two subjects with higher rates of MHb and Heinz body formation were nonsmokers with in vivo carboxyhemoglobin (HbCO) levels of 4–6%. The third subject with low rates of MHb and Heinz body formation was a smoker with in vivo HbCO levels of 15–18%. Increasing levels of HbCO from 8.2% to 14.3% by the in vitro addition of CO caused a marked reduction in the rate of Heinz body formation and a moderate decrease in MHb formation. Rates of MHb formation similar to those observed in normal red cells occurred at HbCO percentages of 89.2 and 99.2. Red cells containing Hb Zürich are extremely sensitive to oxidative injury by PAP, either in vivo or in vitro. The degree of oxidative injury diminishes as the level of HbCO increases, a phenomenon that is enhanced by preferential binding of CO to the abnormal β subunit of Hb Zürich.
Glycophorin a expression in malignant hematopoiesis
Authors: Liszka Kristof , Majdic Otto , Bettelheim Peter , Knapp Walter 
Published: February 2007

AbstractTwo hundred twenty‐nine patients with hematopoietic malignancies were tested for reactivity with a monoclonal anti‐human glycophorin A antibody. One hundred twenty‐three of these cases were classified as acute leukemias of either the myeloid, lymphoid, erythroid, or undifferentiated type. The monoclonal antibody we used (VIE‐G4) was obtained after immunization with a human thymocyte suspension. It selectively reacts with glycophorin A (GpA) and strongly binds to 40% of K‐562 cells and all morphologically recognizable erythroid precursor cells. Apart from two cases with acute erythroid leukemia, this antibody reacted with none of the malignant cells in the 229 tested hematopoietic malignancies, including the 121 nonerythroid acute leukemias. This finding seems to contradict the earlier observations by L. Andersson and colleagues that a considerable proportion of acute leukemias express GpA on their surface. One reason for this discrepancy might be the fact that VIE‐G4 detects only complete glycosylated GpA. If this is the sole explanation, this would mean that the poorly differentiated cells in these cases express incompletely glycosylated GpA.
High‐dose cytosine arabinoside for acute nonlymphocytic leukemia
Authors: Cantin Guy , Brennan James K. 
Published: February 2007

AbstractEighteen patients with acute nonlymphocytic leukemia (ANLL), aged 17–73 years, were treated with high‐dose cytosine arabinoside (HD‐Ara‐C)) using 3 g/m2 IV q 12 hours × 12 doses. Seven patients were treated for relapse and four (57%) obtained a complete remission with a median duration of 19.5 weeks. In nine patients, refractory to conventional chemotherapy, no complete responders were observed. Treatment failure was most commonly due to drug resistance. Two elderly patients with ANLL not previously exposed to chemotherapy died during the initial induction. Recent data on the HD‐Ara‐C experience in ANLL are presented and compared with this study.
Juvenile features in adult‐type chronic granulocytic leukemia
Authors: Gay James C. , Dessypris Emmanuel N. , Roloff James S. , Lukens John N. 
Published: February 2007

AbstractPhiladelphia chromosome (Ph1) positive, or “adult‐type,” chronic granulocytic leukemia (CGL) differs in many clinical and laboratory aspects from PH1‐negative, or “juvenile,” CGL. Because of these differences, the disorders have been considered two distinct disease entities. We present a child with Ph1‐positive CGL whose disease has several features more characteristic of the Ph1‐negative syndrome. This case demonstrates that the chromosomal anomaly in CGL is not always predictive of phenotypic expression in this disease.
In adult acute nonlymphoblastic leukaemia extended maintenance chemotherapy has no benefit
Authors: Jacobs Peter , Dubovsky D. W. , Wood Lucille 
Published: February 2007

AbstractFifty‐two consecutive and previously untreated adults with nonlymphoblastic leukaemia underwent remission induction chemotherapy with a combination of the epipodophyllotoxin VP16‐213, cytosine arabinoside, and doxorubicin (Adriamycin). Complete remission was achieved in 23 of the 52 patients (44%) by means of a single course of therapy in 20 individuals, two courses in two, and three courses in one; median duration of complete remission was 48 weeks. Failure to achieve remission status was due to primary drug resistance in 13 patients (25%), and adequate trial of therapy was not possible in 16 patients (31%) owing to their late referral and accounts for the low remission rate. Individuals achieving complete remission were randomly assigned to receive either 6 (n = 8) or 15 months (n = 13) of maintenance therapy; respective median survival was 95 and 78 weeks (P > 0.10). These data confirm previously reported results for complete remission induction with this three‐drug combination and fail to show any difference between short (6 months) and long (15 months) maintenance chemotherapy.
Variations in the hereditary patterns of factor VIII complex in a large kindred: Coexistence of hemophilia a with von willebrand
Authors: Tavori S. , Tatarsky I. 
Published: February 2007

AbstractCoagulation studies and clinical observations were carried out in 47 members belonging to three generations in one kindred. Classical Hemophilia A, Von Willebrand (VWD) variants, and normal individuals were revealed in this study.The coexistence of Hemophilia A and VWD in different siblings of the same progenitors indicates the difficulty to distinguish between these two major factor VIII abnormalities as two different traits.A hypothesis based on these findings is elaborated.
Platelet factor 4 release during exercise in patients with coronary artery disease
Authors: Levine Shirley P. , Suarez Augustin J. , Sorenson Richard R. , Raymond Nancy M. , Knieriem Linda K. 
Published: February 2007

AbstractMany recent studies provide evidence that increased platelet activation occurs in a significant number of patients with atherosclerotic coronary artery disease. The mechanisms responsible for this activation are unknown, although there have been studies suggesting a correlation with abnormal lipoproteinemia, acute myocardial infarction, unstable angina, and exercise‐induced myocardial ischemia. We studied 84 patients undergoing standardized treadmill exercise using either a Bruce [N = 63] or symptom‐limited Naughton protocol [N = 21]. In contrast to ten healthy volunteer subjects, the patient group demonstrated a significant increase in plasma concentrations of platelet factor 4 [PF4] between pre‐ and postexercise blood samples confirming earlier reports of exercise‐induced platelet activation and secretion. As with previous studies, however, only a subset of patients demonstrated this response. When the entire group was analyzed for the presence or absence of electrocardiographic ischemic changes and the presence of documented versus suspected coronary artery occlusions, there were no differences noted between groups that explained the variable responses measured. However, there was a significant difference between patient groups when analyzed by whether or not they were being treated with β‐blocking agents. Patients who were being treated with propranolol or one of the longer‐acting β‐blocking agents did not have a significant increase in plasma PF4 following exercise, in contrast to patients who were not β‐blocked. Plasma concentrations of epinephrine, norepinephrine, and lactic acid were measured in 49 patients and all normal subjects. There was no correlation between the changes in plasma PF4 concentrations and any of these three variables, suggesting that platelet activation was not occurring through direct platelet activation by circulating catecholamines. This study provides further evidence that there is a subset of CAD patients with platelet hyperactivity. This is the first time that β‐blockade has been demonstrated to modify this platelet response. The effectiveness of β‐blocking agents in CAD may be in part related to their antiplatelet effect.
Kinetics of indium‐111‐labeled leukemic cells in patients with acute nonlymphocytic leukemia
Authors: Yamauchi Kunihiko , Suzuki Yutaka , Sugihara Masami , Nagao Tadami , Arimori Shigeru 
Published: February 2007

AbstractThe distribution within the body of autologous leukemic cells labeled with indium‐111 oxine was studied in seven patients with acute nonlymphocytic leukemia. The leukemic blood cells initially entered the spleen and liver, and the major site of localization was the former rather than the latter. The majority of the leukemic cells had not left the spleen and liver within 48 hr. Liver radioactivity fell transitorily up to the third hr after the initial rise. The clearance curve of radioactivity from the blood showed a plateau or the appearance of a “hump” from 1 to 5 hr after injection of labeled leukemic cells. These results might reflect recirculation of a portion of the leukemic cells between these organs and the bloodstream. In a patient with acute monoblastic leukemia, OKMl monoclonal‐antibody‐treated monoblasts showed the lowest recovery into the blood and a greater increase of liver than splenic radioactivity at 30 min after injection. These results suggest the removal of damaged cells by the cytotoxic effects of antibody mediated by reticuloendothelial clearance mainly of the liver and others. In one patient with acute promyelocytic leukemia, leukemic cells accumulated in both kidneys, indicating the possible infiltration of these cells. Since indium‐111 oxine stays firmly attached to the cells in spite of the possibility of radiation damaged in a long‐term survey, it seems an ideal label for studying leukemic cell kinetics.
Burkitt cell leukemia following treatment of Hodgkin's disease: Report of a case and review of the literature
Authors: Krikler S. H. , Bain G. O. , Gordon P. A. 
Published: February 2007

AbstractThe development of Burkitt cell leukemia (FABL3) [1] in a patient 7 years after successful treatment of Hodgkin's disease (HD) by combined modality (chemotherapy and radiotherapy) therapy is described. The B‐cell nature of the leukemic blasts is shown by immunologic surface marker studies. While L3 leukemia is very rarely seen following therapy for HD, diffuse lymphoma of small noncleaved cells (LSNC)— resembling the Burkitt tumor—is more common in this setting.
An analysis of the mechanism by which cetiedil inhibits the gardos phenomenon
Authors: Berkowitz Lee R. , Orringer Eugene P. 
Published: February 2007

AbstractEnergy depletion in the human erythrocyte causes a rise in intracellular calcium. This in turn accelerates the transmembrane movement of potassium and chloride, resulting in cell dehydration. This process, known as the Gardos phenomenon, is inhibited by cetiedil. The present study examines the mechanism by which cetiedil inhibits the Gardos phenomenon. The ability of cetiedil to retard the initial step in the Gardos phenomenon, a rise in intracellular calcium, was first tested. Cetiedil did not prevent calcium accumulation. Cetiedil's ability to inhibit anion movement was next evaluated, as cetiedil could appear to be blocking K movement when in fact it was preventing the movement of its accompanying anion. No inhibitory effect on anion movement was seen. Since cetiedil prevented neither calcium accumulation nor anion movement, it must inhibit the Gardos phenomenon by preventing the opening of the K‐specific gate in the erythrocyte membrane. The fact that cetiedil's effect on the Gardos phenomenon could not be removed with repeated cell washing indicates that this effect is irreversible.
Selective loss of the expression of OKT‐4 or leu‐3A defined antigen by 12‐0‐tetradecanoylphorbol‐13‐acetate (TPA) in human lymphoid cells
Authors: Tatsumi Eiji , Piontek Catherine , Sugimoto Tohru , Minato Keisuke , Minowada Jun 
Published: February 2007

AbstractIn ten human malignant T‐cell lines (HPB‐ALL, HPB‐MLT, CCRF‐CEM, RPMI‐8402, MOLT‐4, JM, P12/Ichikawa, TALL‐1, SKW‐3, HUT‐102), the expression of the antigen defined by OKT‐4 or Leu‐3A monoclonal antibody was remarkably decreased after a short incubation (1 hr) with 12‐0‐tetradecanoylphorbol‐13‐acetate (TPA) (1.6 nM). This phenomenon was also observed in all types of cells tested, including two lymphoblastoid cell lines with B‐cell properties (RPMI‐6410 and RPMI‐8392), thumus cells from three children, peripheral blood lymphocytes from four normal individuals, and fresh neoplastic T cells from a case of T‐cell acute lympho‐blastic leukemia (ALL) and a case of T‐cell lymphoma. The presence of puromycin (10 μM), cycloheximide (35 μM), or retinoic acid (1.0 μM), had no effect on the TPA‐induced loss of OKT‐4 defined antigen. Incubation at 4°C or with lidocaine (4 mM) partially prevented this effect of TPA, and the presence of sodium azide (150 mM) did so totally. Recovery of antigen expression occurred 24 hours after cessation of TPA treatment. Comparative study using several phorbols other than TPA showed that this effect accorded with the tumor promotor activity of the compounds, but the phenomenon appears to be at the membrane level, not necessarily as a result of TPA‐induced cellular differentiation.
Comparison of the measurement of surface or total platelet‐associated IgG in the diagnosis of immune thrombocytopenia
Authors: Kelton John G. , Denomme Greg , Lucarelli Ann , Garvey Judy , Powers Peter , Carter Cedric 
Published: February 2007

AbstractPlatelet‐associated IgG (PAIgG) can be measured on intact platelets or following platelet lysis. Measurement of PAIgG following platelet lysis may provide different or additional information compared to PAIgG measured on intact platelets. The PAIgG of lysed platelets represents “total” PAIgG, ie, IgG on the surface of platelets plus any IgG that was inside the platelet. To investigate the clinical relevance of the two types of PAIgG assay we performed a prospective study on washed platelets collected from 47 patients with idiopathic thrombocytopenic purpura (ITP). The PAIgG was measured on intact and lysed platelets using an immunoradiometric assay. Platelet‐associated IgG was 2–3 times higher when measured on lysed platelets from healthy controls or patients with ITP compared to PAIgG measured on the same intact platelets. The higher level of PAIgG observed following platelet lysis was not due to the reactions not achieving equilibrium. Using lysed platelets, PAIgG was elevated on 29 of 47 samples from different ITP patients and elevated in 31 samples when measured on intact platelets.The PAIgG is invariably higher when measured following platelet lysis compared measurements made on intact platelets. Neither technique offers a diagnostic advantage over the other.
Severe congenital neutropenia with unique features of dysgranulopoiesis
Authors: Lightsey Alton L. , Parmley Richard T. , Marsh William L. , Garg Aruna K. , Thomas William J. , Wolach Baruch , Boxer Laurence A. 
Published: February 2007

AbstractCongenital dysgranulopietic neutropenia (CDN) is a recently proposed entity that describes a small subgroup of children with clinically severe neutropenia. We followed and studied a 3‐year‐old girl with neutropenia (<500/mm3) and recurrent severe infections in whom repeated marrow evaluations revealed large (30‐50 μm) multinucleated promyelocytes to polymorphonuclear cells with as many as 4 to 16 nuclei or nuclear lobes, respectively. In addition to the nuclear endoreduplication, ultrastructural and cytochemical evaluation of these cells demonstrated abnormalities in granule genesis and centriole structure. Concomitantly. immunoperoxidase staining indicated that many of the granules were devoid of lactoferrin but not lysozyme. In vitro proliferation studies revealed normal to increased thymidine labeling, normal numbers of colony‐forming cells, and normal colony‐stimulating activity from blood and marrow mononuclear cells, findings consistent with ineffective myelopoiesis. However, serum folate, B12, and lysozyme levels were normal. The nuclear and cytoplasmic abnormalities in this patient result in an extreme example of CDN, distinct from previously described cases.
Hairy‐cell leukemia associated with Hodgkin's disease: A case report
Authors: Vokes Everett E. , Bitter Mitchell A. , Prystowsky Michael B. , O'Reilly William , Golomb Harvey M. 
Published: February 2007

AbstractA 56‐year‐old man who had easy bruising, an enlarged left supraclavicular lymph node, and splenomegaly was diagnosed as having hairy‐cell leukemia. Treatment consisted of splenectomy. Because of progressive lymphadenopathy in the following months, the patient required reevaluation. Examination of a lymph node biopsy specimen now revealed Hodgkin's disease, nodular sclerosis. This is the first pathologically confirmed case of hairy‐cell leukemia coexisting with Hodgkin's disease.
α Globin gene rearrangements in polynesians are not associated with malaria
Authors: Trent R. J. , Mickleson K. N. P. , Wilkinson T. , Yakas J. , Bluck R. , Dixon M. , Liley A. W. , Kronenberg H. 
Published: February 2007

AbstractUsing gene mapping, 16.3% of Polynesians were shown to have α thalassemia. These results are surprising since malaria is not found in Polynesia. Moreover, triplicated α gene rearrangements were identified in a further 7.7%, a frequency not seen in other populations.
Normal human blood density gradient lymphocyte subset analysis: I. An interlaboratory flow cytometric comparison of 85 normal adults
Authors: Marti G. E. , Magruder L. , Patrick K. , Vail M. , Schuette W. , Keller R. , Muirhead K. , Horan P. , Gralnick H. R. 
Published: February 2007

AbstractAn interlaboratory flow cytometric comparison of several commercially available human lymphocyte subset reagents was undertaken in three different laboratories. Fresh Hypaque‐Ficoll purified blood mononuclear cells were stained at 4°C or 22°C. Direct or indirect surface immunofluorescence was carried out at all sites using an EPICS V flow cytometer. Fullbright, 10‐μm fluorescent polystyrene microspheres were used for optical alignment and standardization. A log integral fluorescent histogram gated on forward and right angle scatter was collected on 1–2 × 104 cells for each reagent and the proportion, of positive cell determined for each reagent. With the exception of one reagent, anti‐B1, which showed an approximately twofold variation, all three laboratories showed remarkable agreement. Thus there was no significant difference noted for the following reagents: OKT4, CCT4, Leu 3a, Leu 2a, OKT8, or CCT8. We attribute these findings to the availability of quality reagents, precision instrumentation, and a standard lymphocyte preparation.
Prolonged survival in Richter syndrome with subsequent reemergence of CLL: A case report including serial cell‐surface phenotypic analysis
Authors: Baumann Michael A. , Libnoch Joseph A. , Patrick Catherine W. , Choi Hongyung , Keller Robert H. 
Published: February 2007

AbstractThe development of a large cell lymphoma in a patient with chronic lymphocytic leukemia (Richter syndrome) is associated with a poor prognosis. There is dispute regarding the clonal origin of the large cell component. We described a patient in whom prolonged remission of the large cell component was achieved with combination chemotherapy, followed by subsequent reemergence of the less aggressive CLL clone. Serial cell surface phenotypic data are presented suggesting origin of both histologies from a common B‐cell clone.
Dyskeratosis congenita with hypoplastic anemia: A stem cell defect
Authors: Friedland M. , Lutton J. D. , Spitzer R. , Levere R. D. 
Published: February 2007

AbstractA case of dyskeratosis congenita (DC) with pancytopenia was studied in an attempt to elucidate the cause of the hypocellular bone marrow and pancytopenia. Levels of erythroid hemopoietic stem cells were estimated by clonal cell culture. Results showed a decrease in the number of precursor cells in both the bone marrow and the peripheral blood of this patient.
Concomitant inheritance of α‐thalassemia in β°‐thalassemia/hb e disease
Authors: Winichagoon Pranee , Fucharoen Suthat , Weatherall David , Wasi Prawase 
Published: February 2007

AbstractConcomitant inheritance of α‐thalassemia in patients with β°‐thalassemia/hemoglobin (Hb) E disease was detected by restriction endonuclease DNA mapping. Among 42 patients with β°‐thalassemia/Hb E disease, seven were found to have an α‐thalassemia‐2 haplotype. Of these, five belonged to the rightward or 3.7‐kb type of α‐thalassemia‐2 and the remaining two the leftward or 4.2‐kb type. All the seven patients with α‐thalassemia‐2 haplotype had hemoglobin levels of 7.4 g/dl or above; those without detectable α‐thalassemia had hemoglobin levels both higher and lower than 7.4 g/dI. The latter attended the clinic regularly, the former did occasionally. These findings suggest that concomitant inheritance of α‐thalassemia can alleviate the severity of β°‐thalassemia/Hb E disease. Failure to find α‐thalassemia‐1 haplotype in these patients suggests that concomitant inheritance of α‐thalassemia‐1 with β°‐thalassemia/Hb E might lead to so mild a condition that the individuals do not present clinically. The fact that many patients without a detectable α‐thalassemia haplotype also had hemoglobin levels of 7.4 g/dl or higher suggests that there are additional factors responsible for the mildness of β°‐thalassemia/Hb E disease.
In vitro generation of procoagulant activity by leukemic promyelocytes in reponse to cytotoxic drugs
Authors: Fibach Eitan , Treves Aliza , Korenberg Avraham , Rachmilewitz Eliezer A. 
Published: February 2007

AbstractDisseminated intravascular coagulation (DIC) is a frequent occurrence in acute promyelocytic leukemia (APL), especially after onset of chemotherapy. We have used a human promyelocytic leukemic established cell line (HL‐60) and various other human leukemic cells to investigate the effect of cytotoxic drugs on generation of procoagulant activity (PCA). The results indicate that, unlike normal human peripheral blood monocytes and certain other cell types where PCA induction requires active mRNA and protein synthesis, in HL‐60 cells, compounds such as actinomycin D, puromycin, and cytosine arabinoside and a variety of other cytotoxic agents, induced generation of a potent PCA.Although different in its mechanism of induction, this HL‐60 cell PCA was similar, and may be identical, to mononuclear cell tissue factor. The PCA induction was rapid and preceded the lytic effect of the drugs. It was first detected on the outer cell surface but, following prolonged exposure to the drugs, upon lysis of the cells, it was also found in the extracellular medium.This in vitro effect mimics the development of DIC in patients with APL. The system may, therefore, serve as a model for the study of the cellular and molecular events associated with PCA generation by malignant promyelocytes and DIC occurrence in patients with APL and other malignancies.