bluuebunny/crossref_metadata_2025_split huggingface dataset:
Platelet associated complement components (PAC3c and PAC3d) in patients with autoimmune thrombocytopenia
Authors: Hegde U. M. , Bowes A. , Roter B. L. T. 
Published: March 2008

SummaryPlatelet associated C3c and C3d (PAC3c and PAC3d) were quantitated by enzyme linked assay in 105 patients with idiopathic autoimmune thrombocytopenia (AITP) in whom elevated platelet associated immunoglobulins (IgG and IgM) had previously been documented. Increased levels of complement components were demonstrated in 46 of 105 patients (43.8%). In 11 of these patients, PAC3d alone was abnormal implying that C3b had been inactivated after cleavage by C3 inactivator in vivo. Complement binding was seen in two of 16 patients (12.5%) with raised PAIgG alone, four of 19 patients (21.0%) with raised PAIgM alone and in 40 of 70 patients (57.1%) in whom PAIgG and PAIgM were raised together. This difference was highly significant (P= 0.01). The clinical implication of these findings are discussed.
A sensitive cytochemical staining method for glucose‐6‐phosphate dehydrogenase activity in individual erythrocytes II. FURTHER IMPROVEMENTS OF THE STAINING PROCEDURE AND SOME OBSERVATIONS WITH GLUCOSE‐6‐PHOSPHATE DEHYDROGENASE DEFICIENCY
Authors: Van Noorden Cornelis J. F. , Vogels Ilse M. C. 
Published: March 2008

SummaryA cytochemical method for staining glucose‐6‐phosphate dehydrogenase (G6PD) activity in individual erythrocytes as reported previously has been optimized further by the incorporation of a number of technical improvements. Analysis of the enzyme content in erythrocytes of normal individuals as well as patients suffering from G6PD deficiency in the homozygous and heterozygous forms allows these three categories to be easily distinguished. Considerable formazan production occurs in most erythrocytes of a healthy person and only a small percentage of the cells appeared to be negative. Two cell populations of almost equal size could be discerned in heterozygotes for G6PD deficiency, one completely negative, the other with a variable amount of formazan per cell. Homozygous deficiency leads to a population of negative cells with a few positive ones after staining. It is concluded that a reliable method has been found for analysis of G6PD deficiency in erythrocytes at the single cell level.
Transient expression of a second monoclonal component in two forms of biclonal gammopathy
Authors: Bast E. J. E. G. , Slaper‐Cortenbach C. M. , Verdonck L. F. , Samson W. , Ballieux R. E. 
Published: March 2008

SummaryTwo cases of transient biclonal gammopathy are described, one having an IgGK and an IgAK monoclonal component and another with IgG1K and IgGK monoclonal components. In both of these cases the second monoclonal component gradually disappeared. Anti‐idiotypic antibodies were made against the major monoclonal serum component; in the first case the idiotype of the IgGK clone was not found in the IgAK plasma cells (real biclonal gammopathy) whereas in the second case the idiotypes of the two clones were identical (apparent biclonal gammopathy). The evolution to monoclonal gammopathy is discussed with regard to the existence of common malignant precursor cells in biclonal gammopathy.
Platelet hyperaggregability occurring during prolonged continuous intravenous infusions of prostacyclin analogue ZK 36374
Authors: Yardumian D. A. , Mackie I. J. , Bull H. , Goldstone A. H. , Machin S. J. 
Published: March 2008

SummaryWe describe two patients suffering from vascular problems refractory to conventional treatment, who received prolonged continuous infusion of ZK 36374, a stable prostacyclin analogue. During the infusion, the patients' platelets became progressively refractory to the in vitro inhibitory action of exogenous ZK 36374. During the early stages of the infusions, platelet aggregability studied ex vivo was inhibited, but this effect too diminished progressively as the infusions continued. The platelets of one patient become spontaneously aggregable and hyperaggregable to standard agonists during the last 2 d of his 9 d infusion. This effect was not seen during an initial 10 d infusion in our second patient, who was concurrently receiving indomethacin, a reversible cyclo‐oxygenase inhibitor. However, such hyperaggregability became evident in the platelets of this patient during a second, shorter continuous infusion after cessation of the indomethacin. The hyperaggregability was accompanied in each case by a significant rise in serum thromboxane B2 levels.
Haematological change in sickle cell–haemoglobin C disease and in sickle cell‐beta thalassaemia: a cohort study from birth
Authors: Stevens M. C. G. , Maude G. H. , Beckford M. , Grandison Y. , Mason K. , Serjeant B. E. , Taylor B. , Topley J. M. , Serjeant G. R. 
Published: March 2008

SummaryThe haematological changes in early years following neonatal diagnosis have been observed in representative groups of children with sickle cell‐haemoglobin C (SC) disease, sickle cell‐β+ thalassaemia, and in sickle cell‐β° thalassaemia.Most haematological indices in SC disease were intermediate between previously published values in SS disease and in AA controls, generally being closer to values in normal children. Exceptions were microcytosis which may be genetically determined and a striking elevation of mean cell haemoglobin concentration from age 2 months to 4 years. The combination of a raised MCHC and a lowered MCV is unusual and may be characteristic of SC disease.Features in sickle cell‐β thalassaemia generally differed according to the type ofβ thalassaemia gene. Sickle cell‐β° thalassaemia had lower levels of haemoglobin, MCHC, red cell count, MCV, and higher reticulocytes, most differences being significant before 1 year. No differences between Sβ° thalassaemia and Sβ+ thalassaemia were apparent in HbF levels (which resembled those in SS disease) or in HbA2 levels (which exceeded those in SS disease by 1 year of age).
An ultrastructural study of stored human platelets after washing using prostacyclin
Authors: Read N. G. , Radomski M. W. , Goodwin D. A. , Moncada S. 
Published: March 2008

SummaryThe morphology of washed human platelets stored at 4°C has been examined over a period of 96 h. Platelets prepared using prostacyclin showed good preservation of their internal structure. These platelets showed no signs of activation when stirred in an aggregometer and only small reversible aggregates were formed during storage. In contrast, platelets prepared using a more conventional method, which does not use prostacyclin, showed poor preservation of internal structure. Storage and stirring of these platelets resulted in activation and morphological deterioration. These observations support our previous finding that prostacyclin prolongs the viabilty of washed human platelets in vitro.
Standardization of prekallikrein activator (PKA): the 1st International Standard for PKA
Authors: Kerry P. J. , Curtis A. D. , Thomas D. P. 
Published: March 2008

SummaryA international collaborative study was carried out to establish an international standard for prekallikrein activator (PKA). The candidate material, coded 82/530, was assayed against the Office of Biologics (FDA) PKA reference preparation No. 2 (OoB 2) and the 1st British Reference Preparation for PKA. There was good agreement between participant laboratories on the relative PKA activity of the three preparations. Preparation 82/530 has been established by the World Health Organization as the 1st I.S. for PKA, with an assigned potency of 85 International Units per ampoule.
Prognostic value of B‐cell mitogen‐induced and spontaneous thymidine uptakein vitroin chronic B‐lymphocytic leukaemia cells
Authors: Juliusson Gunnar , Robèrt Karl‐Henrik , Nilsson Bo , Gahrton Gösta 
Published: March 2008

SummaryBlood lymphocytes from 50 patients with chronic B‐lymphocytic leukaemia (B‐CLL) were culturedin vitrowith and without the polyclonal B‐cell activators (PBA) dextran sulphate (DxS), lipopolysaccharide fromE. coli(LPS), and Epstein Barr virus (EBV). Patients with blood lymphocytes that showed a high spontaneous or PBA‐induced3H‐thymidine uptake in 4 d cultures had a significantly shorter therapy‐free survival than patients whose lymphocytes showed a low thymidine uptake. The DxS‐induced cellular thymidine uptake was the most powerful predictor of prognosis. Eighteen patients with leukaemic cells responding to DxS stimulation had a median therapy‐free survival of 17 months and a probability of 5 year therapy‐free survival of <0.1, whereas for 30 patients with DxS unresponsive cells the corresponding figures were >120 months and >0.7, respectively (log rank,P<0.0001). A multivariate Cox's regression analysis revealed that the DxS‐induced leukaemic cell response was of greater prognostic importance than clinical features such as blood counts and staging according to Rai and Binet. Therefore PBA‐induced leukaemic cell thymidine uptake seems valuable in the prediction of prognosis in B‐CLL.
High risk of non‐A non‐B hepatitis after a first exposure to volunteer or commercial clotting factor concentrates: effects of prophylactic immune serum globulin
Authors: Kernoff P. B. A. , Lee C. A. , Karayiannis P. , Thomas H. C. 
Published: March 2008

SummaryAfter a first exposure to factor VIII concentrates, 9/9 British patients treated with U.S.A.‐derived commercial products, and 10/12 treated with British volunteer (NHS) products, developed acute non‐A, non‐B (NANB) hepatitis. Hepatitis following commercial products was more severe, and of shorter incubation. High previous exposure to NHS blood products seemed to prevent NHS but not commercial factor VIII‐induced hepatitis; the latter was also not attenuated by administration of U.S.A.‐derived commercial immune serum globulin (ISG). After a first exposure to NHS factor IX concentrates without ISG, 4/4 patients developed short incubation NANB hepatitis; one also contracted prolonged incubation hepatitis B. One patient treated with ISG and factor IX of proven infectivity did not develop hepatitis, suggesting protection by ISG. Observed differences between concentrates might be attributable to their content of different NANB agents, but dose‐related effects could provide alternative explanations. This data provides a basis for comparative assessment of new products of possible reduced infectivity in only small numbers of patients.
Effects of cetiedil on the oxidative metabolism of activated polymorphonuclear leucocytes
Authors: Kohzaki Hitoshi , Kakinuma Katsuko , Asakura Toshio , Yamakawa Tamio 
Published: March 2008

SummaryCetiedel, α‐cyclohexyl‐3‐thiopheneacetic acid 2‐ (hexahydro‐lH‐azepin‐1‐yl)‐ethyl ester, was found to specifically suppress oxygen uptake by polymorphonuclear leucocytes (PMN) that were exposed to myristate or heat‐killed E. coli. The chemical had no effect on the basal respiration rate of PMN in the resting state. Inhibition of oxygen uptake by cetiedil was proportionate to the degree of inhibition of the generation of O‐2 and H2O2. It was also found that cetiedil suppressed the rate of the phagocytosis by PMN of opsonized oil droplets. Cetiedil had no effect on subcellular NADPH oxidase, an enzyme responsible for the respiratory burst that is activated by the perturbation of PMN plasma membrane with phagocytable particles or stimulators such as myristate. These results suggest that cetiedil affects the trigger mechanism of the plasma membrane to inhibit the activation of NADPH oxidase.
Multiple myeloma in a pair of monozygotic twins: the first reported case
Authors: Judson I. R. , Wiltshaw E. , Newland A. C. 
Published: March 2008

SummaryA pair of monozygotic twins both developed multiple myeloma within a 2 year period. The first patient is alive with stable disease 4 years later, his twin brother died within a year of diagnosis. Monozygosity is well established and in both cases the paraprotein was IgGK. The relative influence of environmental and hereditary factors on the aetiology of myeloma is discussed. We believe this to be the first case of myeloma in twins to be reported.
A monoclonal antibody against a Burkitt lymphoma associated antigen has an anti‐Pk red blood cell specificity
Authors: Fellous Marc , Cartron Jean‐Pierre , Wiels Joelle , Tursz Thomas 
Published: March 2008

SummaryThe blood group specificity of a rat IgM monoclonal antibody (38–13) directed against most EBV‐positive or ‐negative Burkitt's lymphoma was investigated. The target antigen was previously identified as the neutral glycolipid ceramide trihexoside (CTH), a substance which accumulates in red cells from very rare individuals of the Pk phenotype and which appears as a normal intermediate in the biosynthetic pathway of the P blood group antigen (globoside).The 38–13 antibody agglutinated Pk/1 and Pk/2 red cells at 4°C with a very high titre but was inactive against native P1, P2 and p erythrocytes, although a very weak activity was noticed towards papain‐treated P1 and P2 erythrocytes. These results were confirmed by an indirect radio‐binding assay which also demonstrated that the 38–13 antibody reacted with lymphocytes, fibroblasts and EBV‐positive lymphoblastoid cell lines derived from Pk/1 and Pk/2 individuals but not from other donors.These findings demonstrate that the 38–13 monoclonal antibody previously considered as specific of Burkitt cells could be routinely used as an anti‐Pk blood group typing reagent. The mechanism of CTH accumulation in Burkitt cells and Pk red cells is probably different and might be associated respectively with the activation of an α‐4‐galactosyltransferase or the genetic blockage of a β‐3‐N‐acetylgalactosa‐minyltransferase.
Suppression of clonal evolution in two chronic myelogenous leukaemia patients treated with leucocyte interferon
Authors: Talpaz M. , Trujillo J. M. , Hittelman W. N. , Keating M. J. , Gutterman J. U. 
Published: March 2008

SummaryTwo patients with Philadelphia chromosome‐positive (Ph1 +) chronic myelogenous leukaemia (CML) were treated with human leucocyte interferon (HuIFN‐α). Karyotypic changes in addition to the Ph1 chromosome developed in these patients before the start of HuIFN‐α treatment. In one patient the administration of HuIFN‐α resulted in clinical haematological remission and stable suppression of the secondary Ph1 clone. The second patient was in myeloid blastic crisis when given HuIFN‐α. While she was receiving HuIFN‐α, suppression of the blast cell population in the bone marrow occurred. The subsequent cytogenetic changes included a near‐complete suppression of a secondary Ph1 clone of cells carrying a deletion in the short arm of chromosome 7 and partial population of the bone marrow with primary Ph1 clone. These observations suggest a potential role for interferons in altering the progressive course of CML.
Changes in platelet immunoprotein levels during therapy in adult immune thrombocytopenia
Authors: Ball S. , Zuiable A. , Roter B. L. T. , Hegde U. M. 
Published: March 2008

SummaryVery early falls in platelet associated IgG, IgM and C3d were demonstrated following therapy with high dose i.v. immunoglobulin and with fresh frozen plasma. This suggests that the changes in platelet immunoproteins may be a primary event and that interference with antibody binding is a possible early mode of action of i.v. IgG.
Serum crosslinked fibrin (XDP) and fibrinogen/fibrin degradation products (FDP) in disorders associated with activation of the coagulation or fibrinolytic systems
Authors: Hunt Frederick A. , Rylatt Dennis B. , H art Rosemary‐Anne , Bundesen Peter G. 
Published: March 2008

SummarySoluble crosslinked fibrin derivatives (XDP) in serum were determined by enzyme immunoassay utilizing monoclonal antibodies and compared with serum fibrinogen/fibrin degradation products (FDP) assayed by conventional techniques.In healthy subjects and patients with miscellaneous disorders not usually associated with activation of the haemostasis mechanism, mean XDP levels were 45 and 70 ng/ml respectively. However, elevated levels of XDP occurred in conditions commonly associated with intravascular and possibly extravascular activation of the coagulation system. Markedly raised mean XDP values (677–6900 ng/ml) occurred in treated pulmonary embolism, disseminated neoplasia, severe inflammatory disorders and complicated postoperative states, and lesser but significant elevation (mean 150–400 ng/ml) in treated venous thrombosis, uneventful postsurgical states, localized neoplasia, liver disease and symptomatic arterial disease. Levels during initial streptokinase therapy (mean 24 000 ng/ml) fell tenfold as treatment was continued.The degree of XDP elevation over normal values was significantly higher than that of FDP in conditions with a propensity for venous thrombosis (post‐operative states, disseminated neoplasia and inflammatory diseases) than in liver disease, localized neoplasia or patients receiving heparin therapy for venous thromboembolism.
Agarose gel method: its usefulness in assaying factor VIII inhibitors, evaluating treatment and suggesting a mechanism of action for factor IX concentrates
Authors: Coots Macie C. , Glueck Helen I. , Miller Mary Ann 
Published: March 2008

SummaryPlasmas from 14 patients with factor VIII inhibitors, 10 haemophiliacs and four non‐haemophiliacs, were assayed by both the agarose gel and Bethesda methods. Good correlation was observed in 34 samples from 13 patients, but there was poor correlation in three samples from a single haemophilic patient. The sensitivity of the method was increased by diluting normal platelet rich plasma (PRP) with congenital factor VIII deficient plasma. With this modification, as little as 0.4 of a Bethesda unit (Bu) could be measured accurately. The agarose method is easier to perform and requires much less technician time than the Bethesda assay (Ba). Inhibitory activity can be measured even in the presence of large amounts of transfused factor VIII or factor IX concentrates. To study the effect of factor IX concentrates on the inhibitor, the method was modified by incorporating into the gels plasmas specifically deficient in either factor VII, IX or X. Our data suggest that factor VII is probably responsible for the ‘bypassing’activity of factor IX concentrates.
Heterogeneity of erythropoietin‐dependent erythrocytosis: case report in a child and synopsis of primary erythrocytosis syndromes
Authors: Kulkarni Vivekanand , Ritchey Kim , Howard Donald , Dainiak Nicholas 
Published: March 2008

SummaryTo investigate the pathogenesis of polycythaemia in a child with isolated, primary erythrocytosis, we measured serum erythropoietin activity and in vitro erythroid progenitor cell responsiveness to erythropoietin. Unstimulated erythropoietin activity was markedly elevated (1.8 IU/ml), and isovolaemic phlebotomy induced a four‐fold increment above this level. In contrast to findings in our index case with this syndrome, normal erythroid colony growth patterns were present in patient marrow cultures. The primary mechanism of polycythaemia in this individual is similar to that reported in the index case: an inappropriately elevated regulatory set point for erythropoietin production. Since an additional defect of progenitor cell hypersensitivity to erythropoietin is not always present, we conclude that abnormalities at single or multiple sites of the erythropoietic regulatory axis may occur in primary erythropoietin‐dependent erythrocytosis.
Treatment of acute lymphoblastic leukaemia in adults
Authors: Barnett M. J. , Greaves M. F. , Amess J. A. L. , Gregory W. M. , Rohatiner A. Z. S. , Dhaliwal H. S. , Slevin M. L. , Biruls R. , Malpas J. S. , Lister T. A. 
Published: March 2008

Summary.Between 1972 and 1982, 112 consecutive previously untreated adults (aged 15–69 years, median 26) commenced therapy for acute lymphoblastic leukaemia (ALL) at St Bartholomew's Hospital. The first 63 patients entered into the study received initial treatment which comprised four cycles of adriamycin and vincristine, prednisolone and L‐asparaginase with the first cycle (OPAL). In 1978, six cycles were given, with escalating doses of adriamycin and cyclophosphamide from cycle 3 (HEAV'D). Central nervous system (CNS) prophylaxis incorporated intrathecal methotrexate and cytosine arabinoside with cranial irradiation. Maintenance chemotherapy consisted of 6‐mercaptopurine, cyclophosphamide and methotrexate for 3 years.Results obtained with the OPAL and HEAV'D regimens were not significantly different. The overall complete remission (CR) rate was 66% (73/111), factors correlating unfavourably with achievement of CR being advanced age (P<0.001) and L3 morphology/B‐ALL immunophenotype (P<0.01). Fifty‐three patients have relapsed, the bone marrow being the primary site in 43. Extramedullary relapse alone occurred in 10 (seven CNS, two testicular and one skin). Only three of the 64 patients who had complete CNS prophylaxis subsequently relapsed in the CNS as an isolated site. One patient died in CR, 19 remain in continuous CR between 2.5 and 10.5 years. The median duration of remission of the 73 patients who achieved CR was 18.5 months, factors correlating favourably with duration of CR being low blast cell count at presentation (P<0.002) and common ALL immunophenotype (P<0.04). Twenty‐four patients remain alive, with a median survival of all patients of 18 months.
Multiple chromosomally distinct cell populations in myelodysplastic syndromes and their possible significance in the evolution of the disease
Authors: Mecucci Cristina , Rege‐Cambrin Giovanna , Michaux Jean‐Louis , Tricot Guido , Van den Berghe Herman 
Published: March 2008

Summary. Serial chromosome investigations performed in untreated myelodysplastic syndromes (MDS) revealed that: (a) multiple chromosomaily unrelated cell populations may emerge during the course of the disease; (b) chromosome changes of the clonal type may disappear or become undetectable, and may or may not reappear during further evolution. The former of these phenomena was found in four cases and was consistently associated with clinical‐haematological progression of the disease to a more malignant form, including acute leukaemia. The second was apparently unrelated to clinical and haematological changes in the evolution pattern.These observations are in favour of the hypothesis that MDS evolve as a multistep process in which several events, including the acquisition of sometimes unrelated chromsome changes determine the progression of the disease.
Studies on immunological assay of vitamin K dependent factors
Authors: Mikami S. , Tuddenham E. G. D. 
Published: March 2008

Summary. We describe and compare five assay systems for Protein C (PC) in human plasma; a functional assay for PC activity, Laurell electroimmunoassay with EDTA or calcium (EDTA‐Laurell or Ca‐Laurell), radioimmunoassay (RIA) and immunoradiometric assay (IRMA). The lower limit of sensitivity of PC in normal reference plasma was 2 × 10−3 units/ml by RIA and 1 × 10−4 units/ml by IRMA, but the ratio of maximum binding to blank binding was superior in RIA.In normal plasma (= 20), there was no significant discrepancy of PC levels between RIA, Laurell (EDTA− and Ca−) and functional assay. In normal serum, PC antigen (PC:AG) measured by Ca‐Laurell showed a high value (1.37 0.24 units/ml) compared to the other assays. PC:AG of a purified sample increased its level by Ca‐Laurell method during activation by thrombin. Activated PC (PCa) has decreased affinity for anti‐PC rabbit IgG, similarly to decarboxy (warfarinized) PC.In warfarin‐treated individuals (= 12) who were anticoagulated orally for more than 3 weeks, functional activity was lower (0.27 0.12 units/ml) than that measured by RIA (0.64 0.12 units/ml) or EDTA‐Laurell (0.62 0.06 units/ml), whereas PC:AG measured by CA‐Laurell had a normal value of 0.96 0.40 units/ml. After stopping administration, the activity showed a gradual increase to the normal at 2 weeks, while PC:AG increased more rapidly to normalize in 1 week.